hematology test 3

what is distribution or dilution thrombocytopenia?

Distribution or Dilution Splenic sequestration Massive blood transfusion Other: Pseudothrombocytopenia Platelet satellitosis Platelet clumping

what are the physical manifestations of disorders of secondary hemastasis?

Bleeding into joints (hemarthoses) Bleeding into deep tissues: peritoneum, body cavities, muscle, central nervous system Bleeding with surgery and dental procedures

what is the difference in lichen sclerosis and lichen simplex chronicus?

in lichen sclerosis- there is a thin layer of hyperkeratinosis, THINNED epidermis and sclerosis of the dermis with atrophy of the adhexa in lichen simplex chronicus= there is dermal inflammation with THICKENED epidermis ad hyperkeatinosis

what occurs in the bone marrow with active hematopoiesis?

- site of ertyhroblastic activity -erythroblast differentiate into red vells -sinusoids, small blood vessels -red cells migrate through endothelium and into vasculature -megakaryoctes give rise to platelets

what does a active thymus look like?

-Active thymus Basophilic cortex Lighter, eosinophilic medulla

what are the factor defs for secondary?

-Factor VII Deficiency Prolonged PT, normal PTT -Factor V, factor X, hypofibrinogenemia, dysfibrinogenemia Prolonged PT, prolonged PTT -Factor XII deficiency Prolonged PTT, Normal PT Asymptomatic! WHY?

where is hemoglobin coded for?

-Globin structural genes for β, γ & δ on chromosome 11, and for α on chromosome 16; gene expression is co-dominant -Globin chain production occurs in ribosomes of nucleated RBC and reticulocytes; mature RBC lack mRNA

what is gestational trophoblastic disease?

-Hydatidiform mole Complete (when 2 sperm meet with an egg that has no maternal DNA or when a X sperm meets an egg with no maternal DNA) the sperm DNA is only duplicated Partial - triploid (69, XXY or XXX) when a 23 X or 23Y sperm meets a 23X egg Invasive mole -Choriocarcinoma

how does hematopoiesis occur in fetal development?

**All blood cells are formed from a common stem cell **hematopoiesis starts in the yolks sac, then the liver, then after birth the bone marrow** Developmental and differentiation processes are controlled by cytokines and local cell/cell interactions Adults have ~1.7 L of bone marrow containing ~10^12 hematopoietic cells On average, ~10^11 blood cells enter the circulation daily (most RBCs, but includes WBCs) Erythropoietin (EPO) is the main controller of red cell production

what is an intraductal papulloma?

**Present with serous or bloody nipple discharge** Ductogram reveals intraductal neoplasm Usually benign** Surgical excision is curative

what is the cause of increased destruction thrombocytopenia?

-Increased Destruction: Immune-Mediated Idiopathic thrombocytopenic purpura (ITP) -antibodies (treat with steroids) TTP/HUS - defect or absence of ADAMSTS13 Drug-Induced - production of antibodies - withdraw drug Heparin induced thrombocytopenia (HIT) - withdraw heparin Neonatal alloimmune and autoimmune thrombocytopenia Post-transfusion purpura (thromobocytopenia) Non-Immune Mediated: Pregnancy- accelareated activation and consuption Pre-eclampsia/eclampsia/HELLP syndrome-vacular damage and platelet activation HIV- multifactorial- infected Megakaryocyte, AIDS related infection, AIDS related drug, and sometime AIDS related ITP. Disseminated intravascular coagulation (DIC): causes thrombosis and bleeding Sepsis, surgery, trauma, pregnancy/childbirth, burns, etc. Mechanical (prosthetic material)

what are breast infiltrating carcinoma's?

-Infiltrating Ductal Carcinoma -Infiltrating Lobular Carcinoma (Similar clinical presentations Histologic type determined by microscopic examination) prognosis: Determined by characteristics of tumor Histologic type Size Grade (nuclear grade, tubule formation, and mitotic count) Lymphovascular invasion Lymph node metastases Distant disease Estrogen, progesterone, and HER2-neu receptor status treatment: Complete surgical excision External beam radiation therapy +/- Chemotherapy +/- Tamoxifen (anti-estrogen therapy) Breast reconstruction

what is found in the thymic medulla?

-Inner part of the parenchyma -Contains large numbers of epithelioreticuar cells and loosely packed T-cells -Stains lighter because the T-cells are bigger & less packed

what is a choriocarcinoma?

-Malignant neoplasm derived from gestational chorionic epithelium or from totipotential cells -Often associated with history of complete mole or abortion *****-β-HCG levels are extremely elevated Metastases are common to lungs, vagina, brain, liver, and kidneys

what is pseudomyxoma peritonei?

-May result from rupture of primary mucinous ovarian tumors *****-More likely from metastasis from GI tract, primarily from appendix (Krukenberg tumor)***

what are the diseases of pregnancy?

-Preeclampsia / Eclampsia -Placental Inflammation and Infection -Ectopic Pregnancy -Gestational Trophoblastic Disease

what is preeclampsia/ecampsia?

-Preeclampsia is characterized by Proteinuria Edema HTN -May progress to eclampsia (seizures)

what are the normal CBC ranges?

-RBC Count-4. -5 million/ microliter Hemoglobin-14-16 g/dl Size-7.5 microns Life Span -120 days -WBC Count-4,000 - 10,000/cubic mm -Platelets Count -150,000-400,000 /cubic mm Life span -7 - 10 days

impaired production thrombocytopenia is caused by:

-Reduced megakaryopoiesis: Aplastic Anemia -Due to drugs, chemical, radiation, idiopathic -Marrow infiltration Tumor, infection, fibrosis -Congenital Congenital amegakaryocytic thrombocytopenia (autosomal recessive), Thrombocytopenia with absent radius (TAR) syndrome (autosomal recessive), Wiskott-Aldrich Syndrome, Faconi Syndrome -ineffective megakaropoiesis: vitamin b12 deficiency - Alcohol suppression - Myelodysplasia

can cervical cancer be caused by CIN?

-Risk factors for HPV and CIN -Early age at first intercourse -Multiple sex partners -Male partner with multiple previous sex partners -Persistent infection by high-risk HPV -HPVs 16, 18, 31, 33, 35, 39, 45, 52, 56, 58, and 59

what is breast carcinoma?

-Second leading cause of cancer death in US women -Risk Factors Females Increasing age Family history -Potential Risk Factors: Prolonged exposure to HRT Oral contraceptives Radiation Obesity EtOH consumption Fatty diet -Noninvasive Carcinoma Ductal carcinoma in situ (DCIS) Lobular carcinoma in situ (LCIS) Paget's disease -Invasive (Infiltrating) Carcinoma Infiltrating ductal carcinoma Infiltrating lobular carcinoma Others

what are the mucinous tumors of the ovary?

-Similar to serous tumors except: Larger, multilocular, Epithelium consists of mucin secreting cells in the Endocervical and Intestinal area -Papillary formations less common -No psammoma bodies 5-20% bilateral 10% malignant 10% low malignant potential

what is a phyllodes tumor?

-True fibroepithelial neoplasm -Tend to be large -Leaflike clefts and slits -Prognosis depends on histologic grading -Most are localized and cured by complete surgical excision

what is a blood smear?

-commonly stained with Wright's stain, a mixture of dyes to detect cytoplasmic granules -based on how they stain you can determine what types of cells are present in the blood

how does the neonatal alloimmue thrombocytopenia occur?

-during delivery, the mother is exposed to the fetus's HPA-1a positive patelets, the - after delivery:mother creates anti-HPA1a antibodies from - subsequent pregnancy= anti-HPA1a antibodies from the mother attack and destroy the HPA-1a positive platelets in the fetus

what do eosinophils do?

-have large bi-lobed nuclei and large specific granules that stain red with eosin -Granules contain ***peroxidase*** which binds to microorganism and facilitates their killing -**Major basic protein (MBP) binds** to and disrupts membranes of parasites; causes basophils to release histamine -Eosinophils accumulate in blood and tissue in response to parasitic infection -Increased numbers of eosinophils in allergic reactions such as hay fever -They have surface receptors for IgE - the antibody seen mostly in allergic responses

summary of the thymus: The primary lymphoid organs (BM, Thymus) are responsible for the development of immunocompetent B and T cells in the absence of antigen. Stromal cells play an important role in the maturation process. Immunocompetent cells are delivered to the secondary lymphoid organs by lymph and blood. Activation of both B and T cells in the presence of antigen takes place in the peripherial (secondary) lymphoid organs

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what is the presentation of a squamous cell carcinoma in men ?

0.25% of cancers in men; patients > 40 years of age Rare among circumcised men Associated with sexually transmitted (HPV) types 16 and 18; similar to cervical squamous cell carcinoma in women -Tumor is usually locally invasive and indolent ~25% metastasize to regional lymph nodes

classification of platelet -related bleeding disorders?

1. Quantitative (common) Thrombocytopenia 2. Deficiency/dysfunction of platelet interactors Vascular disorders (rare) von Willebrand Deficiency (common) 3.Disorders of platelet receptors (rare) Bernard-Soulier Syndrome Glanzman's Thrombasthenia 4.Disorders of platelet granules (rare) Dense granule deficiencies α-granule deficiency 5.Exogenous inhibition of platelet function (common) Liver disease Renal disease Drugs (aspirin)

how do the lymph nodes, B cell activation?

1. antigen 2. initial T-cell and B-cell activation 3. primary follicle= B cell activation 4. B-cell proliferation and differentiation 5.plasma-cell secretion of antibody 6. efferent lymphatic vessel

what is the mechanism of clot formation?

1. vessel wall injury 2. vessel wall contraction 3. platelet adhesion 4. platelet aggregation 5. fibrin formation

how are the secondary lymphoid organs organized?

1.Diffuse collections of lymphocytes and macrophages (lung and lamina propria of intestinal wall- MALT/GALT) 2.Lymphoid follicles (aggregates of lymphoid cells, stromal cells, macrophages surrounded by a network of lymphoid capillaries) 3.Lymph nodes (highly organized secondary lymphoid organs containing several lymphoid follicles with distinct regions of T and B cell activity) 4.Spleen (highly organized with distinct regions of B and T cell activity)

how do Hematopoietic Cells mature?

1.Pluripotent stem cells - give rise to ALL types of blood and lymphoid cells 2.Multipotent stem cells - lymphoid and myeloid lineage 3.Committed progenitor cells (colony forming cells) - lymphoid, erythroid 4.Precursor cells (blasts) - undergoing structural differentiation into specific lineage 5.Mature cells (erythrocytes, lymphocytes, megacaryocytes)- megacaryocytes give rise to platelets

what are the 2 major parts of the lymphoid system?

1.Primary lymphoid organs - Bone marrow - Thymus 2. Secondary lymphoid organs - Lymph nodes - Lymphatic nodules - MALT - GALT - Spleen - Skin associated

what is hemoglobin C disease?

2-3% of African Americans Much milder than Sickle Cell Disease No pain crises, mild hemolytic anemia, splenomegaly Usually, no treatment is necessary Lower oxygen affinity

what are lymhocytes?

30-40% of total WBC Two sizes, large ~9-12mm (NK), or small ~6-8mm (B & T) Spherical nucleus, slightly indented Cytoplasm stains lightly and appears as a small rim around the nucleus. Three classes of lymphocytes: B-lymphocytes T-lymphocytes NK cells Lymphocytes in blood are circulating immunocompetent cells They have a capacity to recognize and respond to antigens B-cells can mature into Ig producing plasma cells - in secondary lymphoid organs

how many erythrocytes are in a cubic mm?

4.2-6.2 million they make up 99% of the cells in the blood

what is the lifetime risk of HPV?

75% population has it 50% exposure to high risk HPV 10% persistent high grade CIN 1.3%invasive carcinoma 0.4%death

what is the clinical significance of thombocytopenia?

<10,000 transfuse prophylactically in th absence of the bleeding <20,000 transfuse if there is a significant risk of bleeding <50,000 transfuse if actively bleeding, undergoing surgery, or in an invasive procedure

what are the pathological hemoglbins?

> 893 identified, most have no physiologic significance Name alphabetically, geographically or a combination i.e Hgb G, Hgb O, Hgb Wennipeg, Hgb O arab, etc 90% have due to amino acid substitutions, usually affecting alpha or beta chains; other chains (i.e. gamma, delta) can be affected but not usually significant due to low concentration of hgb of these type in blood Point substitution of base codon Amino acid substitution Hgb S (β6 Glu >Val) Hgb C (β6 Glu >Lys) Hgb S and C are most prevalent in Africa and people of African descent

what are alpha platelet granules?

Alpha-Granules: contain PDGFs which is released at the site of a wound and initiates surrounding cells to undergo mitosis.

what is the tonsil?

A circular "ring" of lymphoid tissue guards the entrance to the nasopharynx and oropharynx - termed Waldeyer's Ring there are 4 tonsils in the oropharynx area torus tubarius contains the tubal tonsils, pharyngeal tonsils (adenoids), the palatine tonsils, and the lingual tonsils

what is a venereal disease?

A lengthy list of pathogens, including bacteria, viruses, and protozoans, encompasses most of the sexually transmitted disease. We will focus, however, on only few of these pathogens Neisseria gonorrheae Treponema pallidum Herpes genitalis HPV

what is blood?

A specialized connective tissue. Adults have ~5-6 liters of blood. RBCs and platelets remain in the vascular system. WBCs may leave the circulation. Only lymphocytes can return to the vascular compartment.

what is ernard-Soulier Syndrome

Bernard-Soulier Syndrome (adhesion and aggregation) Rare, autosomal recessive Deficiency of Gp-Ib (Gp-Ib/V/IX binds vWF) Gp-Ib also involved in binding other molecules Platelet dysfunction Giant platelets on peripheral smear Usually severe Diagnose with platelet aggregation studies- decreased response to ristocetin

Which of the following is a granulocyte? A. T cell B. Macrophage C. Platelet D. Neutrophil E. B cell

A. T cell B. Macrophage C. Platelet Correct! D. Neutrophil The correct answer is D. Granulocytes include neutrophils, basophils, and eosinophils. T cells are formed from myeloid stem cells and are this considered myelogenous in nature. Platelets are formed from the breakdown of magakaryocytes. B cells are formed from lymphoid cells and are thus lymphocytes. E. B cell

Contents of Platelet Dense Granules

ADP (activates neighboring platelets) ATP Calcium Serotonin

what is dysfunctional uterine bleeding? DUB?

Abnormal bleeding in the absence of a well-defined organic lesion Causes: Anovulation Inadequate luteal phase Contraceptive-induced bleeding Endomyometrial disorders

what is the main difference in the fetal and adult/newborn hemoglobin?

Adult haemoglobin is made up of four subunits. The most common form, Hb A consists of two alpha and two beta subunits (α2 β2). However a second similar but less abundant form is also found, Hb A2, with two delta subunits replacing the beta (α2 δ2) During pregnancy, the mother is responsible for delivering oxygen to the baby, and removing CO2. In order for this to happen, maternal and fetal circulations come into very close proximity in the placenta. It is important that this system preferentially moves oxygen into fetal circulation and CO2 into maternal circulation. This would not happen if both circulations had the same oxygen transporter with the same binding characteristics as the gases would bind equally between the two circulations. To overcome this, mammals have evolved a unique form of haemoglobin for fetuses, known as fetal haemoglobin (Hb F) consisting of two alpha and two gamma subunits (α2 γ2). This allows Hb F to bind oxygen with a higher affinity than adult haemoglobin (Hb A), allowing extraction of oxygen from maternal circulation. This results in a left-shifted haemoglobin-oxygen dissociation curve compared to adult haemoglobin. This means oxygen loading occurs at lower oxygen concentrations.

what are the normal types of hemoglobin?

Adults (A): Hgb A1 (alpha2Beta2): 95-98% α & β chain synthesis starts in second month of gestation and (α > β rapid synthesis). Adult A Hb increases rapidly after birth (>95% by 3-6 months after birth) Hgb A2 (alpha2delta2) : 2-3% δ chain synthesis begins shortly before birth. Normal adult proportion (2.5 - 3.2%) is reached by 3-6 months after birth Child (Hgb F): newborn: 50-80% 6 months: 8% greater than 6 months: 1-2% Hgb F (alpha2,gamma2): 0.8-2.0% α2 γ2 γ chain around first month of gestation and rapidly increases and accounts for 70-85% of the newborn Hgb. Normal 1% (adult level) is reached by 3-6 months after birth. Hgb F has slightly higher affinity for oxygen than Hgb A

what does the bone marrow contain?

All T- and B- lymphocytes, mononuclear phagocytes, platelets, erythrocytes, and other leukocytes originate from the bone marrow (BM) in adults -The ****multipotent stem cell (CFU-Ly) divides to form CFU-LyB, and CFU-LyT -BM stromal cells interact with B cells and produce cytokines that govern B cell development -Self reactive B cells are eliminated in the bone marrow -Immunocompetent B-lymphocytes develop in the BM, and travel to the secondary lymphoid organs. -Antigenic specificity of mature, immunocompetent B cells is represented by the single antigen specific Ig they express on their surface (IgM, IgD) -Immuno-incompetent T cells travel to the thymus where they further develop

what are some functional hemoglobin abnormalities?

Amino acid substitution at globin chain interfaces alter solubility of Hgb molecule Hgb S = Deoxygenated form is rigid / forms tactoids (crystals) that deform RBC shape (sickled RBC) > hemolysis and veno-occlusion Hgb C = Forms rhomboid crystals, target cell; hemolysis, usually mild

what is a nongonococcal urethritis?

Among the most common STD Pathogens: Chlamydia trachomatis, Ureaplasma urealyticum, Mycoplasma hominis Presentation similar to gonococcal urethritis -Cervix involved in women May occur simultaneously with gonococcal infection Treatable with antibiotics (doxycycline or azithromycin)

what is endometritis?

Associated with Retained products of conception IUD use Tuberculosis Pelvic inflammatory disease (PID) Spontaneous Chronic endometritis has plasma cells, lymphocytes, and macrophages

what is an endometrial polyp?

Benign sessile endometrial projection into uterine cavity that may cause uterine bleeding Common at menopause

what is a leiomyoma (fibroid)?

Benign smooth muscle tumors Estrogen stimulates growth May cause pelvic pain and bleeding

how the in vitro coagulation assays work?

Blood collected in 3.2% sodium citrate Sodium citrate acts by binding and sequestering calcium which is an important cofactor in many of the coagulation cascade reactions Ratio of blood to anticoagulant is critical (9:1) (4.5 ml blood added to 0.5 mL of sodium citrate) Prompt processing of sample necessary High hematocrit >55%, falsely prolongs testing Avoid contaminating with Heparin

what are the primary lymphoid organs?

Bone marrow: (and fetal liver) serve as B cell development zones Thymus: development of naive T cells, T cell receptor gene rearrangement, selection Function In the development and maturation of lymphocytes into mature, immunocompetent cells

what is thrombosis?

Can occur as a result of congenital or acquired conditions. Risk factors for thrombosis include age, immobilization, diet, hyperlipidemia, oral contraceptives, pregnancy, hormone replacement therapy, long bone fractures, hip, knee, gynecologic, and prostate surgery, smoking, inflammation, and placement of a central venous catheter Disorders predisposing to thrombosis Antiphospholipid syndrome Malignancy Patients can present with deep venous thrombosis, pulmonary embolism, retinal thrombosis, mesenteric vessel thrombosis, lost pregnancies

what is the structure of the thymus?

Capsule and trabeculae contain blood vessels, efferent (but not afferent) lymphatic vessels, and nerves. In addition to collagen fibers and fibroblasts, the connective tissue of the thymus contains variable numbers of plasma cells, granulocytes, lymphocytes, mast cells, adipose cells, and macrophages. The trabeculae establish regions in the thymus called thymic lobules.

what is placental inflammation and infection?

Caused by an ascending infection that affects: Chorionic Membranes Placental Villi Umbilical cord Fetus Associated with premature rupture of membranes, preterm labor, and fetal death in utero

what are the alpha thalassemias?

Caused by deletions in the α chain Alleles (α) normal or "wild-type" (-) absent α chain DNA The α chain on chromosome 16 is duplicated This means rather than having 2 copies of the gene, each individual has a total of 4 copies, two from each parent Normal αα/αα Silent Carrier α-/αα α Thalassemia Minor α-/α- αα/-- α Thalassemia Major α-/-- Hydrops Fetalis --/--

what is the life span of the platelet (thrombocyte)?

Cell fragments, derived from megakaryocytes in the bone marrow. Discoid shaped Life span ~10 days Contain granules - contents are involved in the blood clotting

what are the 2 types of cellular growth in the cervix?

Cervicitis (acute and chronic) Cervical Intraepithelial Neoplasia (CIN I-III)

what are the values you want to check for secondary homeostasis deficiencies?

Commercial Thomboplastin varies between labs -> INR developed to standardize values PT and PTT

what occurs in a UTI?

Common Infection of the lower urinary tract (Bladder, prostate, urethra) E. coli, Proteus, Klebsiella, Enterobacter (culture for diagnosis and to determine therapy) More common in females in absence of instrumentation or obstruction Fever, dysuria, frequency Risk of developing pyelonephritis

what are fibrocystic changes?

Common finding, especially during reproductive years -Palpation reveals ***discrete lumps -Dense -Smooth surface -Mobile (not fixed to surrounding tissue) -May be sore or painful to touch -Nonproliferative Cystic Lesions Cyst formation Stromal fibrosis Apocrine Metaplasia Mild hyperplasia Fibroadenoma -Proliferative Lesions Moderate to florid hyperplasia (without atypia) Ductal papillomatosis Sclerosing Adenosis Atypical hyperplasia (ductal or lobular)

what is an inguinal hernia?

Common in pediatric population, but can occur at any age Loop of intestine protrudes into the tunica vaginalis through the inguinal canal Can distinguish from other masses by the presence of bowel sounds Painful May become incarcerated Treated surgically

what are some of the secondary defs?

Congenital Deficiences Hemophilia A (common) Hemophilia B (common) Hemophilia C (rare) Factor VII Deficiency (rare) Factor V Deficiency (rare) Factor X Deficiency (rare) Factor XII Deficiency (rare) Hypofibrinogenemia, dysfibrinogenemia (rare) Acquired Deficiencies Liver disease (common) Vitamin K deficiency (common) Disseminated intravascular coagulation (DIC) (common)

what is the cause of thrombosis?

Deficiency in any of Protein C, Protein S or antithrombin can lead to a prothrombotic state. Antithrombin deactivates factors II, IX, X, XI, XII Homozygous deficiency incompatible with life Can be acquired with liver disease, nephrotic syndrome, etc. Protein C (enzyme) and Protein S (cofactor) together act to deactivate factors V and VIII Activated protein C resistance (Factor V Leiden) Prothrombin 20210 mutation

what is the thymus?

Epithelial in origin -outgrowth of the third pharyngeal pouch T cell maturation Antigenic diversity of T cell receptors is generated by gene rearrangements Programmed cell death - thymocyte selection (apoptosis, 95-99%) -Lies posterior to manubrium & extends into superior & anterior mediastinum Largest and most active during neonatal and pre-adolescent periods. By early teens, thymus begins to shrink (thymus involution) and thymic stroma is replaced by adipose (fat) tissue.

how does O2 dissociation occur?

Definitions: O2 pressure- oxygen tension or partial pressure of oxygen in blood O2 saturation- proportion of hemoglobin its saturated form or the ratio of oxygen bound to hemoglobin to hemoglobin's oxygen binding capacity. Purpose: To measure how readily hemoglobin acquires and releases oxygen in the surrounding fluid/tissue First oxygen molecule is difficult to bind, with following molecules more easily bound. Eventually approximate limit is reached at o2 pressure of 60 mmHg Shift right- harder to bind, easier to release Shift left- easier to bind, harder to release P50-partial pressure in blood in which oxgen saturation is 50% (usually 26.6 mmHg. Certain conditions disease changes oxygen affinity, shift curve left or right and changes p50 (p50 increases with right shift, decreases with left shift) 2,3 BPG, H, CO2, and Atp bind the central cavity and alter O2-Hb affinity (right or left shift). 2,3 BPG is an organophashate created in RBCs during glycolysis, usually occurs in O2 depleted states, i.e anemia, COPD, fetal hemoglobin Clinical scenarios: Exercise- Tissue such as muscle needs oxygen, needs a situation where oxygen more likely to be release, right shift. Exercise leads to increase temp., lactic acid production (H+), CO2 production Newborn- More hemoglobin F, which has higher binding affinity, left shift

what are delta granules?

Delta-Granules: release SEROTONIN, HISTAMINE, ATP and ADP; facilitates platelet adhesion and causes vasoconstriction in the area.

what is the pathology of syphilis?

Destructive vasculitis with plasma cells seen in all three phases Tertiary disease: Gummas (Granulomas) with necrotic centers and surrounding vasculitis Treatment: Penicillin

what is Hypospadias:

Developmental Abnormalities Hypospadias: The urethra opens anywhere along the underside of the penis or perineum

what are some disorders of vasculature?

Disorders of the vasculature are rare 1.Genetic Marfan Syndrome: disorder of fibrillin Ehlers-Danlos Syndromes: disorders of collagen Many others... 2. Acquired Vitamin C deficiency (scurvy) Vitamin C necessary for the production of collagen

what is DIC in secondary?

Disseminated Intravascular Coagulation Caused commonly by sepsis but also burns, trauma, malignancy, host of other causes In sepsis DIC is caused by intravascular coagulation triggered by foreign organisms in the bloodstream Intravascular coagulation (in undamaged vasculature) causes thrombosis of end-organs Uncontrolled clot formation depletes coagulation factors from the circulation resulting in bleeding Very difficult to treat because of the combination of bleeding and thrombosis

what is the fibrinolytic system?

Dissolves clot: Plasminogen converted to plasmin by tissue plasminogen activator, breaking down fibrin and dissolving clot Medication such as streptokinase activates this pathway for patient with clots.

what is a neoplasia of the penis or urethra?

Dysplasia, condyloma Squamous cell carcinoma in situ (Bowen's Disease/erythroplasia of Queyrat) Precancerous lesion Associated with human papilloma virus (HPV) types 16 + 18 (and other high risk HPV)

what is sarcoma botryoides?

Embryonal rhabdomyosarcoma Rare primary neoplasm Children less than 5 years of age

tumors of the endometrium and myometrium?

Endometrial polyp Leiomyoma Leiomyosarcoma Endometrial stromal tumors Endometrial carcinoma

what is Epispadias:

Epispadias: The urethra opens anywhere along the dorsum of the penis

what are M cells?

Epithelial cells that overlie Peyer's patches Take up microorganisms and macro-molecules from the lumen in endocytotic vesicles. Antigen-transporting cell. The vesicles are transported to the basolateral membrane where they discharge their contents in the vicinity of CD4+ T cells.

what occurs in exogenous inhibition of platelet function?

Exogenous inhibition of platelet function (platelet aggregation) May be present when platelet count is normal but patient has bleeding Many causes (common): Aspirin and many other drugs Liver or renal failure -Not well understood why. Herbs and excess vitamins Tested in the laboratory by Platelet Aggregation assay

what is vaginal clear cell adenocarcinoma? what causes it?

Occurs in teenage girls whose MOTHERS took diethylstilbestrol (DES) during pregnancy Risk is 1:1000 exposed in utero Vaginal adenosis

what is pagets disease?

Extension of malignant cells along lactiferous ducts to the skin Invariably associated with underlying ductal carcinoma, either DCIS or invasive Prognosis is determined by histopathology of the primary lesion

what is a prostatic adenocarcinoma?

Extremely common malignancy, about 200,000 new cases/year (50% by 80 years) ****Second leading cause of cancer-related mortality for men Etiology is unknown Presentation variable, dysuria, decreased stream or retention of urine. Bone pain in patients with bony metastases. -Pathology: Adenocarcinomas of varying degrees of differentiation Gleason's grading system provides five grades, from least to most differentiated May extend locally to involve seminal vesicles, bladder, or rarely rectum Metastasizes via lymphatics and hematogenously *****Bone is most common site (ribs, spine, pelvis): Typically causes new bone formation (Osteoblastic) Prognosis correlates well with combination of Staging (based on size and extent of tumor, lymph node status, and metastasis) and histologic grade Tumor marker prostate specific antigen (PSA) commonly used as screening test for diagnosis, and to follow patient up for recurrences after treatment Treatment depends on stage and histology but may include surgery, and/or radiation Orchiectomy ("endocrine therapy") performed and estrogens given in advanced disease. Based on proposed androgen dependence of tumor cells.

what are risk factors of an endometrial carcinoma?

Presents with postmenopausal bleeding Risk factors Obesity Diabetes HTN Infertility (increased estrogen exposure) Staging is surgical Prognosis: Depth of myometrial invasion Extension to adjacent tissue Peritoneal washings (Peritoneal washing is a procedure used to look for malignant cells, i.e. cancer, in the peritoneum. Peritoneal washes are routinely done to stage abdominal and pelvic tumours, e.g. ovarian cancer.) Lymph node metastases

what is a monocyte?

Fairly large single nucleated cells. Shape of the nucleus is often indented or horse-shoe shaped Monocytes circulate in the blood ~3 days and then migrate into the tissues When monocytes leave the circulation and enter the tissue they differentiate into macrophages - comprise the mononuclear phagocytic system. Cells phagocytose bacteria, cells and tissue debris. ****Also express MHC II molecules.

what is the spleen?

Filters blood and responds to systemic infections. Blood born antigens and lymphocytes are carried through the splenic artery. -The spleen is enclosed by a dense connective tissue capsule from which trabeculae extend into the parenchyma containing myofibroblasts. The parenchyma of the spleen is the splenic pulp. The splenic pulp is divided into two functionally and morphologically different regions: white pulp & red pulp.

lymph nodes

Small encapsulated organs located along the pathway of lymphatic vessels Range in size from 1 mm-2 cm Serve as filters through which lymph percolates on its way to the bloodstream

what is benign prostatic hyperplasia?

Generally > 50 years; increasing incidence with age. (20% by 40 years and 90% by 80 years)

what is glanzmann's thrombasthenia?

Glanzmann's Thrombasthenia (platelet aggregation) Rare, autosomal recessive Deficiency of Gp-IIb/IIIa (binds fibrinogen) Usually severe Platelets are normal sized Diagnose with platelet aggregation studies-decreased response to all platelet agonists except ristocetin

what are pathologies in male breasts?

Gynecomastia Enlargement of male breast due to relative estrogen excess Carcinoma Extremely rare Usually advanced disease at presentation, but, stage for stage, same prognosis as female breast cancer May have positive family history (BRCA-2)

what is HPV?

HPV types 6 and 11 Causes squamous proliferation in genital tract Condyloma acuminata Appears as papillary proliferations up to several cm in diameter -Microscopically appear as exuberant proliferation of squamous epithelium supported by fibrovascular papillae Superficial epithelial cells contain irregular, hyperchromatic nuclei surrounded by a clear perinuclear halo (koilocytosis)

what are the types of hemophilia?

Hemophilia A: X-linked Congenital deficiency of factor VIII Prolonged PTT, normal PT Hemophilia B: (Christmas Disease) X-linked Congenital deficiency of factor IX Prolonged PTT, normal PT Hemophilia C: (Rosenthal Syndrome) Autosomal dominant Congenital deficiency of factor XI Prolonged PTT, normal PT

how does heparin work?

Heparin Heparin is often used in hospitalized patients when initiating warfarin Overlap of Heparin with Warfarin allows time for all Vitamin K dependent factors to decrease (bridging) Heparin acts by potentiating the effect of antithrombin Recall antithrombin inhibits II, IX, X, and XI Monitor heparin with the PTT (sensitive to factor changes in factor XI) Low Molecular Weight Heparin Direct factor 10a inhibitors Direct thrombin inhibitors

what is hemoglobin S/beta thalassemia?

Hgb S/ β thalassemia Third most common sickling disease behind SS and SC in African Americans, most common in Mediterranean. Severity depends on if β, β+, or β0 Sickle cells are pretty rare on the blood smear

what is hemoglobinS/alpha thalassemia?

HgbS/ α thalassemia Sickle cells uncommon Anemia less severe, but vaso occlusive crisis is still very severe

what is a seminoma?

Histologic differentiation towards germ cells (looks like sperm precursors) Peak ages 40 to 50 years Characterized by rapid growth Metastasizes relatively late via lymphatics, in contrast to Nonseminomatous GCT's ***Good prognosis as tumor is particularly radiosensitive, even metastases cells look like fried eggs

what is a Yolk Sac Tumor (Endodermal Sinus Tumor)?

Histologically differentiated towards extraembryonic membranes (yolk sac) Most cases in children age ≤ 3 years Uniformly expresses AFP -Schiller-‐Duvall Body—Has Central Vessel

what is a chriocarcinoma?

Histologically differentiated towards placenta Highly vascular with predilection towards hemorrhage Peak ages 20-30 years ****Metastasizes early, especially to lungs and liver***** May present as metastatic disease, when testicular mass is small

what is an embryonal carcinoma?

Histologically poorly differentiated Peak ages 20-30 years Express AFP, and/or hCG, though not uniformly; consistent with pluripotential tumor

how do you grade Cervical Intraepithelial Neoplasia?

Histology CIN I: Mild dysplasia CIN II: Moderate dysplasia CIN III: Severe dysplasia and carcinoma in situ

how do we evaluate primary hemostasis?

History Duration of bleeding Inherited or acquired Family history Spontaneous or precipitated by surgery or trauma Location and type of bleeding Physical Exam Mucocutaneous: petechiae, purpura, ecchymosis. Petechiae - tiny pinpoint hemorrhages that occur on skin and mucosa Purpura - skin or mucosal bleeding larger than a petechiae Ecchymosis - large and severe bruise following minimal trauma. Mucous membranes: menorrhagia (increased menstrual flow), epistaxis (nosebleeds), gastrointestinal bleeding.

what is a hydrocele?

Hydrocele Most common cause of scrotal swelling Tunica vaginalis (mesothelial-lined sac/extension from peritoneum) collects serous fluid from trauma or generalized edema In infants, usually resolve. Easily corrected surgically

how does T lymphocyte differentiation occur?

Immuno-incompetent cells enter the medulla via postcapillary venules and then migrate to the cortex TCR develops through migration and meeting with self antigens Positive and negative selection Mature, immuno-competent cells leave through the post capillary venules

what is a Nonseminomatous Germ Cell tumor?

In Contrast with Seminoma, metastasize early via hematogenous routes Not radiosensitive, requires more vigorous chemotherapy Tumors markers, though not specific for these tumors, are used to aid in diagnosis and follow up for recurrences after therapy

testicular germ cell tumors occurs when?

Incidence of 2/100,000 Peak ages 15-34 years Typically presents as painless enlargement of testicle Germ Cell Tumors (GCT's) represent about 95% of all testicular tumors Tumors arise from pluripotential cells and may differentiate along different lines within the same tumor or even when they metastasize. <40 % are single tumor type

what is orchitis?

Infection of the testis, less common than epididymis Most common causes: Mumps, T. pallidum, tuberculosis, brucellosis, and autoimmune form Viral orchitis is uncommon cause of sterility in adult males.

what is epididymitis?

Infection usually secondary to UTI or prostatitis In < 35 years age group, N. gonorrhoeae and C. trachomatis most common pathogens

what is the role of the specific (adaptive) immunity?

Initial contact with a specific antigen or foreign agent initiates a chain of reactions involving effector cells (B and T cells) and frequently leads to state of immune "memory" Results in acquired resistance Adaptive immunity includes: 1.)Humoral responses resulting in the production of antibodies that mark destruction of invaders by other cells 2.)Cellular immune response targeting transformed cells and virus infected cells for destruction by Killer T Cells. 1.Antigenic specificity (distinguish subtle differences between antigens) 2.Diversity (generation of diverse recognition molecules) 3.Immunologic Memory (increased state of reactivity at the second encounter with the same antigen, lifelong immunity to infectious agents) 4. Self/nonself recognition (immune response to foreign antigens, tolerance to self molecules)

what does an involuted thymus look like?

Involuted thymus Atrophic lobules, cortex Large Hassal's corpuscules (arrows) Fat tissue

what is hassal's corpuscle?

Isolated masses of closely packed Type VI epithelioreticular cells Contain keratohyalin granules Bundles of intermediate filaments Center may be keratinized

what are some of the key immune components of the blood?

Key components of blood: Albumin (most abundant protein in the plasma) Immunoglobulins (IgA, IgG, IgM, IgE, IgD), Complement system Lipoproteins; chylomicrons, VLDL, IDL, LDL, HDL Plasminogen, transferrin, and other important transport proteins. Fibrinogen and other clotting molecules Hormones (soluble or protein bound) Electrolytes, nutrients, gases, etc.

what are NK cells?

Lack of B or T cell specific markers Lack of specific antigenic receptors Recognize altered cells (tumor, virus infection) Recognize Ig constant region (Fc receptor, CD16) ADCC (antibody dependent cell-mediated cytotoxicity) - NK cell binds to cell surface through surface antigen -macrophage has antigen bound to Fc receptor -eosinophil and neutrophil come bind to antigen -all 4 cells release different substances from their granules-ultimately leads to destruction of target cells

how are the liver and vitamin K involved in secondary defs?

Liver Disease and Vitamin K deficiency The liver produces factors II, VII, IX, X in a vitamin K dependent manner Liver disease or vitamin K deficiency will cause a decrease in these factors The PT is most sensitive because VII is at the beginning of the in vitro assay

what are peyer's patches?

Located in the ileum and consist of numerous aggregations of lymphatic nodules

where are the palatine tonsils located?

Location: at the boundary of the oral cavity and the palatopharyngeal folds Structure: the bases are encapsulated, surfaces are covered by oral epithelium, crypts

where are the lingual tonsils located?

Location: posterior third of tongue Structure: covered with stratified squamous epithelium, mucous salivary glands open at the bottom of a crypt\ B in the picture

where are the pharyngeal tonsils (adenoid) located?

Location: roof of the nasal pharynx Structure: thin capsule, shallow infolds (pleats), ducts of mixed glands open to the base of pleats. covered by pseudostratified, ciliated, columnar and stratified squamous epithelium.

what are the secondary lymphoid organs?

Lymph Nodes: filter lymph Spleen: filters blood MALT/GALT: first defense against external antigens

what are the lymphatic system vessels?

Lymphatic vessels begin as networks of blind capillaries in loose connective tissue. These vessels remove substances and fluid (lymph) from the extracellular spaces of the connective tissues. The walls of the lymphatic capillaries are permeable - large molecules, including antigens & cells enter. Lymph passes through lymph nodes. Within the lymph nodes, antigens are trapped then presented by APCs within the lymph node

what are the types of macrophages?

Macrophages = phagocytes -In liver -Kupffer cells -In bone marrow - osteoclasts -In skin - histiocytes -In lungs - alveolar macrophages -In the nervous system - microglial cells

what are neutrophils?

Main phagocyte of the blood Very motile cells Multi-lobed nucleus and two types of granules - 1.)Primary granules—contain elastase and myeloperoxidase 2.) Azur granules (secondary)---lysozyme and proteases Have large stores of glycogen, few mitochondria Circulate for ~10h then migrate to tissue Body's first line of defense vs. bacteria

what is a leiomyosarcoma?

Malignant smooth muscle neoplasm High metastasis rate 40% 5 year survival

what is crytorchidism?

Malpositioning of testes along embryonic pathway of descent into scrotum Majority not associated with an obvious anatomic abnormality Majority unilateral, 25% bilateral Results in atrophy of affected testicle during childhood -May result in infertility, even if second testicle normally positioned *****Clinical importance: Increased risk of cancer in both descended and undescended testes (10 to 40 fold higher) Surgical correction (orchiopexy) does reduce the risk of sterility and cancer.

what is LCIS? lobular carcinoma in situ

Marker of increased risk and a possible precursor Requires no treatment Close follow-up of bilateral breasts is recommended

what is methemoglobinemia?

Methemoglobinemia- iron in hemoglobin is converted from ferrous (Fe2+) to ferric (Fe3+) state, with higher binding affinity, left shift

what is hemoglobin SC disease?

Mild hemolytic anemia Crisis less frequent and less painful the sickle cell anemia. Onset usually childhood but can be later Life expectancy only modestly shortened Hip pain due to aseptic necrosis of the head of femur Target cells and sickle cells on a peripheral blood smear

what is the structure of hemoglobin?

Molecule composed of 4 globin chains, each with 1 heme group. Newborn / adult α:141 amino acids β:146 amino acids γ:146 amino acids δ:146 amino acids Fetal ε:Embryonic chain ζ:Embryonic chain

what occurs in carbon monoxide poisoning?

More clinical scenarios: Carbon Monoxide poisoning- CO has high hemoglobin binding affinity, harder for O2 to be released, left shift

serous tumors of the ovaries?

Most frequent Age 30-40 Solid or cystic Tall columnar epithelium Psammoma bodies 25% bilateral 25% malignant 15% low malignant potential

what is the function of the white pulp?

Mostly lymphocytes Central Artery Periarterial Lymphatic Sheaths (PALS): lymphocytes aggregated around the central artery Splenic nodules - enlarged germinal centers for B-cell expansion Sinusoids

what is adenomyosis?

Nests of endometrial glands and/or stroma extend from surface deep into the myometrium (ie. Think of it as "Endometriosis within the uterus") May cause menorrhagia, dysmenorrhea, or pelvic pain

what is the difference in beta minor and beta major thalassemia?

Normal β/ β Thalassemia Minor β+/ β β0/ β Clinical:Microcytic anemia, Mild Hemolyti, Anemia Increase HbA2 (alpha2delta2) Thalassemia Major β+/ β+ β0/ β+ β0/ β0 Clinical: Microcytic anemia, Severe transfusion-dependent anemia, Presents in childhood, Increased HbA2 (alpha2delta2) and HbF (alpha2gamma2) Mechanism Decreased β chain production relative to α chain Decreased overall production of hemoglobin/red cells causes microcytic anemia Excess α chain in remaining red cells precipitates and causes increased red cell destruction If destroyed while still in marrow contributes to decreased production If destroyed after release in circulation causes hemolysis

what is a teratoma?

Occurs at all ages May metastasize if malignant transformation present in any component (e.g. squamous cell carcinoma in skin-like areas) Metastatic potential more often expressed in adults; therefore, all teratomas treated as malignant in adults -Histologically differentiated towards all three primitive layers of embryonic tissues: Ectoderm (neural and skin) Mesoderm (muscle, cartilage, fat) Endoderm (gut and lung)

how do you diagnose syphilis?

Organism cannot be cultured. Rely on serologic tests for antibodies. Non-treponemal (VDRL & RPR). Venereal disease research laboratory (VDRL) Rapid plasma reagent (RPR) Detect antibodies against host tissues (heart, lipid) Treponemal (FTA-abs & MHTPA)(More specific) -Fluorescent Treponema antibody-absorption test -Microhemagglutination-Treponema pallidum VDRL or RPR Positive only 75-84% in primary 100% in Secondary and Tertiary May be positive in other infections or autoimmune diseases (not specific) Disappears with successful treatment Specific test (FTA = fluorescent Treponemal antibody) Detects antibody to organisms, Used to confirm positive VDRL or RPR, Antibodies may remain even with cure

what is endometriosis?

Outside of the Uterus (ovary, peritoneum, paratubal areas, etc) Characterized by any 2 of 3 features Endometrial glands Stroma Hemosiderin pigment Can cause: Infertility , Dysmenorrhea, Pelvic Pain

what is the function of hemoglobin?

Oxygen transport (O2), bound to hemegroups Four heme groups sequentially bind O2 altering conformation of the Hb molecule "heme-heme interaction" resulting in progressive decrease of O2 affinity (# 3/4 hem groups deliver O2 to the tissues) Sigmoid shaped O2-Hb disassociation curve pO2 100 mm = 100% saturation (lung) pO2 40 mm = 66% saturation (tissues)

what is herpes gentialis?

Pathogen: Herpes Simplex Virus types I and II -Genital infection more commonly with type II in US -Type I more commonly associated with orolabial lesions (cold sores & fever blisters) Clinical Presentation: Multiple painful blisters that rupture and ulcerate on external genitals, cervix in women Keratitis (corneal infection) may cause ulcers and scarring Lesions last about 1 month, then resolve Primary infection-more lesions plus local lymphadenopathy and systemic symptoms-fever, malaise headache, though may be asymptomatic Lesions recur periodically Transplacental or transvaginal transmission serious consequences for neonate Pathology: Characteristic multinucleated epithelial giant cells with intranuclear Cowdry Type A viral inclusions (Tzanck smear)

what is syphilis?

Pathogen: Treponema pallidum Increasing incidence of infection Chronic disease with symptomatic and asymptomatic intervals Clinical course: 3 phases primary: Chancre (ulcer) at site of infection with local lymphadenopathy Appears in first few weeks, heals spontaneously secondary: Develops about 6-8 weeks after primary stage. Organisms disseminate via blood stream Diffuse or patchy red-brown rash on skin and mucosal surfaces Involvement of palm and sole Majority remain asymptomatic, no disease progression. Even if lesion free and asymptomatic, patients may remain infectious. May have generalized lymphadenopathy. Plaque (condyloma lata) on anogenital region. Relapse of secondary disease typically within 1st year, 1/3 of patients spontaneously cured tertiary: 1/3 progress to this stage at 1-30 years post infection Signs and symptoms highly variable, making diagnosis difficult CNS and cardiovascular, and bone most common sites of disease manifestations ***Pathologic lesion is "Gumma" - nodular granulomatous lesion that rarely demonstrates organisms.

what is phimosis?

Phimosis: Constriction of the foreskin over the glans penis such that it cannot be retracted back; predisposes toward infection and can lead to URINARY RETENTION. -May be developmental or acquired (most common secondary to inflammation

what is benign nodular hyperplasia (Benign Prostatic Hypertrophy)?

Presents with difficulty starting and maintaining stream of urine ****Significance: Urinary tract obstruction which can promote urinary tract infection or hydronephrosis** No increased risk of cancer Treated medically or surgically

how do we diagnose primary hemostasis?

Platelet count most important determinant Included in the complete blood cell count (CBC) Low count - thrombocytopenia - most common cause of bleeding -Platelet function: vWF function/activity Platelet aggregation

what are the contents of platelet alpha granules?

Platelet specific proteins: (indicate platelet activation when present in serum) Platelet factor 4, ß - thromboglobulin, basic protein. Coagulation factors: Fibrinogen, factor V, von Willebrand factor. Glycoproteins: Thrombospondin, fibronectin, albumin Growth factors: Platelet-derived growth factor (PDGF), transforming growth factor -ß (TGF-ß), connective tissue activating peptide Other substances: Immunoglobulin G, osteonectin

in beta thalassemias what happens to the mRNA?

Point mutation in β chain causing unstable mRNA (β chain only) - decreased or absent transcription of mRNA Alleles β normal or "wild-type" β+ decreased β chain synthesis β0 absent β chain synthesis One β chain sequence inherited from each parent Each individual has two (potentially) different β chain sequences, one on each of two copies of chromosome 11

what is the nonspecific (innate) immune response?

Preexsting nonspecific defenses against microbial invasion Consists of: 1.Physical barriers (skin, mucous membranes 2.Chemical defenses (low pH) 3.Secretory substances (lysozymes, thiocyanate in saliva, interferons) 4.Phagocytic cells (macrophages, neutrophils, monocytes) 5.Natural killer (NK) cells

what are dendritic cells?

Present antigens to T helper cells Exhibit long membrane extensions Location: Langerhans cells : epidermis and mucous membranes Interstitial dendritic cells: heart, lungs, liver, kidney GI tract Interdigitating dendritic cells: T cell areas of secondary lymphoid tissues, thymus Circulating dendritic cells: blood, lymph

what is a T lymphocyte?

Present membrane receptor for antigen. Recognize antigens presented with MHC molecules on the surface of cells (APC, virus infected cells). Effector cells: - T helper (TH,CD4+,cytokines) - T cytotoxic (TC , CD8+ , CTL) T cells function in Immunologic Memory

inflammation in the breast presents as:

Present with breast tenderness, skin erythema, and breast edema Palpable mass may or may not be identified -Mastitis:Usually associated with history of breastfeeding Treat with antibiotics -Duct ectasia: Occurs in women in 40s or 50s Produces skin / nipple retraction -Traumatic fat necrosis

how does a transitional cell carcinoma present?

Presentation: Painless hematuria May cause outflow obstruction in bladder or ureters Flank pain may be present in renal pelvis tumors Unifocal or multifocal lesions ("field effect") -Low-grade urothelial tumors ****Always papillary Noninvasive, but up to 50% may recur (or new primaries) EXCELLENT prognosis (98% 10-year survival) -High-grade urothelial carcinoma ***Papillary, flat or both Considerable cellular pleomorphism and anaplasia *****80-90% recur Lethal in 60% of cases within 10 years of diagnosis -All urothelial cell tumors, whatever the grade, tend to recur following excision. Prognosis depends on the grade (histologic pattern) and stage (extent of spread) when first diagnosed

what is a Non-Neoplastic -Epithelial Disorders-Vulvar Dysplasia?

Presents as leukoplakia Differential diagnosis Psoriasis Lichen planus Carcinoma in situ Invasive carcinoma Paget disease Need a biopsy to confirm diagnosis

what is primary hemostasis?

Primary Hemostasis Vasculature and Platelets Acute bleeding, oozing / Mucosal surfaces

what can cause endometrial hyperplasia?

Proliferation of endometrial glands secondary to relative estrogen excess

why is the blood-thymus barrier important?

Protects developing T-cells from antigens 1.Capillary endothelium & BM 2.Macrophages in perivascular connective tissue 3.Type I epithelioreticular cells

what is DCIS? ductal carcinoma in situ

Requires complete surgical excision with subsequent external beam radiation therapy Prognosis is generally excellent

what is testicular torsion?

Results from trauma to the spermatic cord which carries neurovascular supply to testes Usually underlying structural abnormality allows excess mobility ***Intensely painful Venous stasis leads to extravasation of blood, and subsequently hemorrhagic infarct if untreated

what is the Reticular Meshwork?

Reticular cells: synthesize and secrete type III collagen and the matrix that forms the stroma Elongated processes for isolating the structural components from the parenchyma Dendritic cells: APCs that monitor for foreign particles and present them to T-cells Macrophages: phagocytic & antigen-presenting (less than DCs) Follicular dendritic cells: have multiple, thin, hairlike cytoplasmic processes that interdigitate between B-cells in the germinal centers Antigen-antibody adhere to their processes via Fc receptors; they lack MHC II molecules

what is a risk factor for ovarian tumors?

Risk Factors Nulliparity Family history (BRCA genes) Patients usually present with advanced disease

what are the risk factors for transitional cell carcinoma?

Risk factors: Cigarette smoking Chronic bladder infection Industrial solvent ß-naphthylamine Cyclophosphamide (chemotherapeutic agent)

what is the splenic sinus?

Special sinusoidal vessels lined by rod-shaped, loosely-packed endothelial cells Macrophage processes extend between them to monitor passing blood for foreign antigens Have only strands of basal lamina which loop them at right angles to the endothelial cells No smooth muscle & no pericytes

what pathologies can occur in the Fallopian tubes?

Salpingitis (Pelvic Inflammatory Disease) Ectopic Pregnancy Endometriosis Paratubal cysts

what is secondary hemostasis?

Secondary Hemostasis Coagulation Cascade Delayed bleeding / Large wounds, deep sites, joints, surgery, dental procedures

what re the functions of the secondary lymphoid organs?

Secondary lymphoid organs capture antigens and provide sites where lymphcytes undergo clonal proliferation and differentiation into effector cells in the presence of antigens.

what is plasma that lacks the coagulation factors?

Serum

what is sickle cell disease?

Sickle trait (AS) one normal β chain, one β6 Glu >Val Asymptomatic 8% of African-Americans, 45% of Africans; also seen in Mediterranean, Middle East, and India Most common hemoglobinopathy in US Sickle cell disease (SS) Both maternal and paternal copy are β6 Glu >Val 1 in 375 live African-American births Median age of death is in 40's Patients suffer from vaso-occlusive episodes Acidosis, hypoxia, dehydration, infection, fever, cold Vaso-occlusion causes: Pain crises (sickling in bones) Acute chest syndrome Hepatomegaly, jaundice Autosplenectomy - results in infection (Salmonella, pneumococcus), sepsis, osteomyelitis Priapism Hemolytic anemia, aplastic anemia High risk pregnancy Treatment with transfusion, red cell exchange, and hydroxyurea

what is the difference in alpha major and minor thalassemia?

Silent Carrier: No symptoms/normal labs α Thalassemia Minor: Mild microcytic anemia (due to decreased production), Mild or no hemolysis α Thalassemia Major (Hemoglobin H Disease) Microcytic anemia (due to decreased production); Southeast Asians Moderate hemolysis with mild transfusion requirement Presence of Hgb H (β4) HgH precipitates resulting in hemolysis Hydrops Fetalis --/-- is incompatible with life; stillborn severe edema and marked anemia In intrauterine life production of Hgb Bart (γ4) Hgb Bart has extremely high oxygen affinity and therefore does not unload oxygen to the tissues effectively resulting in intrauterine fetal demise

___and ___ decrease the occurrence of malaria

Silent carriers of sickle cell disease disease and thalassemia are essentially asymptomatic and experience a milder form of malaria Due to selective pressure, in malaria-endemic areas (Africa and Mediterranean) these mutations survived despite their severe manifestations in individuals with multiple copies of the genes

what do Erythrocytes look like? what is their function?

Small 7.8 mm biconcave disk shaped cells; extremely pliable. Circulating RBCs lack nuclei & organelles***. Almost all the protein found in the RBC is hemoglobin. Glycolytic enzymes are also present to generate ATP. Life-span of the RBC is approximately 120 days. Old cells are phagocytosed by macrophages in spleen, liver and bone marrow. Function to transport oxygen from lungs to tissues; and to pick up CO2 from tissues and transport it to lungs.

what is meant by the phrase "splenic open circulation"?

Splenic artery ->white pulp via central artery Central artery -> white pulp & sinuses at the perimeter called marginal sinuses. Continues into the red pulp -> branches into penicillar arterioles. Some penicillar arterioles are surrounded by aggregations of macrophages and are called sheathed capillaries. Sheathed capillaries then empty directly into the reticular meshwork of the splenic cords rather than connecting to the endothelium-lined splenic sinuses. Here they are sampled by macrophages before reentering the circulation through the splenic sinuses

what is the function of the red pulp?

Splenic sinuses Splenic cords (cords of Billroth): consist of loose reticular cells & fibers; RBCs; dendritic cells, plasma cells; granulocytes & macrophages that phagocytose damaged RBCs, releasing iron to be used in the formation of new RBCs.

storage pool defienceies of primary hemostasis:

Storage pool deficienceies (i.e. Granule Deficiencies) Platelet aggregation studies Dense granule deficiency (platelet aggregation) Relatively mild in isolation Exacerbated when patients take platelet inhibitors like aspirin Also seen as a component of other more severe systemic disorders Wiscott-Aldrich Syndrome Chediak-Higashi Syndrome Thrombocytopenia with Absent Radii Syndrome (TAR) Alpha granule deficiency (platelet aggregation): Varies in severity Grey platelets on peripheral smear (due to lack of granules)

what is a B lymphocyte?

Synthesis and display of membrane bound antibodies (IgM & IgD) that function as antigen receptors Synthesis of circulating antibodies - Plasma B cells & Memory B cells

how do the platelets aid in clotting?

Surface receptors on platelets bind collagen IV activates thrombin Collagen binding is followed by aggregation - formation of a plug. Release of granules to activate clotting cascade Formation of a fibrin polymer that seals damaged blood vessel.

Which of the following is TRUE of platelets? A. They range from 200,000 to 400,000 per mm3 blood B. They are involved in immunoregulation C The average lifespan is upward of 120 days D. They are nucleated E. They function in blood gas transport

The correct answer is A. Platelets range in number from 200,000-400,000 mm3 of blood. Platelets are involved with hemostasis and have a life span of 7-10 days. They are involved in blood clotting, not gas transport, and are anucleate.

A 60-year old chronic alcoholic with know alcoholic cirrhosis presents with an upper gastrointestinal hemorrhage. Despite prolonged tamponade, bleeding is detected. A coagulation defect related to the liver is suspected. Which of the following abnormalities is most consistent with this possibility? Correct! A. Deficiency of all clotting factors except von Willebrand factor B. Deficiency of factors II, VII, IX and X C. Deficiency of factors II, V, VII, and X D. Deficiency of factors IX, X, XI and XII E. Deficiency of von Willebrand factor

The correct answer is A. The liver is the site of production of all coagulation factors except von Willebrand factor (vWF) and severe hepatic dysfunction can thus be associated with multiple factor deficiencies, excluding vWF.

A 55-year old woman with chronic pancreatitis undergoes coagulation screening before surgery. The prothrombin time and activated partial thromboplastin time are found to be prolonged. Given the following choices, which of the following is the most likely reason for the abnormal coagulation test results? A. congenitial bleeding disorder B. fat malabsorption and vitamin K deficiency C. Glutamate deficiency due to impaired digestion of dietary protein D. Nutritional vitamin C deficiency E. Post-pancreatic carcinoma of the pancreas

The correct answer is B. Chronic pancreatitis causes fat malabsorption, because lipase is required for fat digestion. Fat malabsorption leads to deficiency of the fat-soluble vitamins A, D, E, and K. Vitamin K is required in the synthesis of clotting factors II, VII, IX, and X and as a cofactor for the conversion of glutamyl residues to gamma-carboxyglutamates.

A 4-year old boy has recurrent joint pain involving the knees and hips. He had always bruised easily and recently his parents have seen blood in his urine. A presumptive diagnosis of classic hemophilia (hemophilia A) is made and coagulation blood tests are performed. Which of the following is the most likely set of findings of the coagulation screening tests? A. normal bleeding time, platelet counts and thrombin time; prolonged prothrombin time (PT) and activated partial prothromboplastin time (PPT) B. normal bleeding time, platelet counts, thrombin time, and PTT; prolonged PT C. Normal bleeding time, platelet count, thrombin time and PT; prolonged PTT D. Normal platelet counts and thrombin time, prolonged bleeding time, PT and PTT E. Prolonged bleeding time, PT, PTT, and thrombin time; decreased platelet count

The correct answer is C. Class hemophilia (factor VIII deficiency) is an abnormality of the intrinsic pathway of coagulation proximal to the final common pathway which begins at factor X to factor Xa activation. This defect leads to a prolonged activated thromboplastin time. The other lad tests listed remain normal because the bleeding time is a measure of platelet plug formation, the prothrombin time a measure of the extrinsic pathway of coagulation, and the thrombin time an assay of the conversion of fibrinogen to fibrin. The presumptive diagnosis is confirmed by specific factor VIII assay.

Which of the following is the site of B-cell maturation? A. lymph nodes B. blood C. Thymus D. Bone marrow E. target tissue

The correct answer is D. Bone marrow is the site of B-cell maturation. This is where pluripotent stem cells become phagocytes or lymphocytes. The lymph nodes are the site of further lymphocyte differentiation as well as lymph filtering. The thymus is the site of T-cell maturation. The target tissue is a site of further lymphocyte differentiation. Immunologic responses also occur here.

A 23-year old African American man with a history since early childhood of severe anemia requiring many transfusions, has non-healing leg ulcers and recurrent periods of abdominal and chest pain. These signs and symptoms are most likely to be associated with which of the following laboratory abnormalities? A. Decreased erythropoietin B. Increased erythrocyte osmotic fragility C. Schisocytes D. Sickle cells on peripheral blood smear E. Teardrop-shaped cells

The correct answer is D. Sickle cell anemia is the most common hereditary anemia of persons of African lineage. Leg ulcers and recurring painful crises are characteristic. In sickle cell anemia, in contract to sickle cell trait, sickle cells are often seen on the peripheral blood smear.

A 14-year old girl presents with prolonged bleeding from wounds and minor trauma as well as severe menorrhagia. Family history reveals that her father also has prolonged bleeding from wounds and minor trauma as does her brother. Which of the following is the most likely mechanism of this patient's disorder? A. Absence of platelet glycoproteins IIb-IIIa B. Antiplatelet antibodies reacting with platelet surface glycoproteins C. Deficiency of factor VII D. Deficiency of factor IX E. Deficiency of von Willebrand factor

The correct answer is E. Von Willebrand diseases, a disorder transmitted by autosomal dominant modes of inheritance (both dominant and recessive) is the most common hereditary bleeding disorder. There are many variants, all marked by deficiency of von Willebrand factor.

Extramammary Paget Disease

The vulva is in the "milk line", therefore tumors typically associated with breast can occur Scattered single intraepithelial carcinomatous cells -Biologically dormant or invades locally with metastases

what are types of thalassemias?

These result from abnormalities of the rate of globin chain synthesis Subnormal Hb synthesis (e.g. microcytosis and hypochromia) Relative excess of unsuppressed chain resulting in precipitations of excess chains and RBC membrane damage > hemolysis Two major types (decreased globin chain synthesis) β Thalassemia (more common) α Thalassemia (also common) Both types are most prevalent in the Mediterranean or people of Mediterranean descent (Greek: θαλασσα = "Thalassa" = sea)

what is a Transitional Cell (Urothelial) Carcinomas?

Transitional Cell (Urothelial) Carcinomas May occur anywhere there is transitional epithelium from calyces to urethra Most commonly found in bladder Less common in ureters and renal pelvis (5% to 10%) > 50 years age, males > females

what is the function of blood?

Transport - oxygen, carbon dioxide, nutrients, hormones, chemical signals Buffer system - maintain acid base balance Temperature control - in conjunction with blood vessels that dilate/constrict to release or conserve heat Removal of cellular and metabolic waste Immune function - defense against infection Coagulation - functions to prevent massive blood loss

what are the 2 major types of bladder carcinomas?

Two major types: Transitional cell (urothelial) carcinoma (90%) Squamous cell carcinoma (5%) Numerous cytogenetic alterations, especially deletions in chromosomes 9, 13 (Rb gene), 14 and 17 (p53 gene) Squamous Cell carcinoma is associated with bladder infection by Schistosoma hematobium.

what is the function of the epithelioreticular cells in the cortex?

Type I: boundary of cortex & capsule or cortex and trabeculae or surrounding cortical blood vessels -separate the thymic parenchyma from the connective tissue of the organ -occluding junctions provide barrier function Type II: within the cortex -Stellate structure -Compartmentalize the cortex into isolated areas for developing T-cells -MHC I and MHC II molecules to aid in thymic cell development Type III: boundary between cortex & medulla -Barrier function -Also express MHC I and MHC II molecules

what are the Epithelioreticular Cells in Medulla?

Type IV: boundary of cortex & medulla aid the Type IIIs with the corticomedullary boundary Type V: within the medulla similar to Type IIs, create compartments Type VI: within the medulla form Hassall's corpuscles; may produce IL-4 and IL-7 for thymic differentiation and education

what is Polycystic Ovaries (PCO)-Stein-Leventhal Syndrome?

Usually affects young women Clinical presentation Oligomenorrhea Hirsutism Infertility Obesity ***High LH and Low FSH

what can cervicitis be causes by?

Vaginal flora Streptococci Staphylococci Enterococci Escherichia coli Clinically significant organisms: Chlamydia trachomatis Ureaplasma urealyticum Trichomonas vaginalis Candida species Neisseria gonorrhea HSV HPV

what is neoplastic growth in the vagina?

Vaginal intraepithelial neoplasia (VAIN) Squamous cell carcinoma Clear cell adenocarcinoma Sarcoma botryoides

what is the presentation of gonorrhea?

Very common, > 1 million new cases yearly in US Peak ages 15-30 years Pathogen is Neisseria gonorrhoeae, a gram negative diplococcus Pathogenesis: Attachment to epithelium using bacterial pili Mucosal invasion and transport across the epithelium to subepithelium --Release of proteases to destroy antibodies normally present in the vaginal and urethral secretions Membrane components have antineutrophil and anti-complement activity Organism elicits intense neutrophilic response, resulting in copious amounts of pus Clinical course: Within first week after infection Suppurative urethritis with discharge Symptoms of dysuria. Cervicitis Intermenstrual bleeding and Vaginal discharge. Anorectal area or pharynx may be site of primary infection in both sexes Diagnosis rests on culture and gram stain of smears of discharge ****If treated at this point, no long term sequelae If untreated, organism may ascend the genitourinary tract to infect the following: Ovaries and Fallopian tubes. Endometrium Prostate Epididymis Disseminated infection via blood stream unusual complication. Skin Joints Heart valves Meninges Eye infection of neonates obtained during passage through birth canal

what are basophils?

Very low number in the circulation, less than 1% of leucocytes of blood Nucleus is typically bilobed Specific granules are large and stain dark blue or purple; contain heparin and vasoactive peptides Basophils play a role in bronchial asthma Have IgE surface receptors which, when bound, trigger release of vasoactive peptides

what is blood made up of?

Volume of cells and plasma is ~45 and 55% respectively Hematocrit: volume of packed erythrocytes in a sample of blood Normal hematocrit: ~39-50% in males ~35-45% in females Leukocytes and platelets constitute only ~1% of blood volume

what are the staged of vulvar tumors?

Vulvar Intraepithelial Neoplasia (VIN I-III) VIN I VIN II VIN III (carcinoma in situ) Invasive Vulvar Carcinoma

how does warfain work?

Warfarin (Coumadin) Warfarin is a vitamin K antagonist Mimics liver disease and vitamin K deficiency by decreasing productions of II,VII,IX, X Monitored by PT (very sensitive to factor VII) Protein C and protein S are also produced by the liver in a vitamin K dependent manner and also decrease when warfarin is started Unfortunately when warfarin is started (presumably to prevent thrombosis) the protein C/S inhibition dominates and there is an initial prothrombotic effect`

what is a fibroadenoma?

Young women Benign neoplasms Discrete well-circumscribed mobile lesions

how long does it take for a erythrocyte to mature?

about 3 days first the pro-eryrthroblasts undergo maturation, then they will increase the amount of hemoglobin present then the loss of organelles and nucleus

how are B lymphocytes activated?

antibodies on cell surface binds to antigen via B cell receptor -if no antibody can recognize antigen it wont bind these cells with help of helper T cells can differentiate into plasma cells (they are also able to release antibodies into the plasma)

what is ehlers-danlos syndrome?

blue sclera

what is thrombocytopenia?

can be caused by : -Impaired production -Increased destruction -Distribution or dilution -Pseudothrombocytopenia

features of the complete an partial hydatidiform mole:

complete: karyotype: 46 XX or XY villous edema: all villi trophoblast proliferation: diffuse, circumferential atypia: often present serum hCG: elevated behavio:; 2%choroca partial mole: karotype: triploid (69, XXY) villous edema: come villi tropholoblast: focal, slight atypia: absent serum hCG: less elevated behavior: rare chorioca

what occurs in the phagocytosis of the neutrophil?

different receptos on cell surface when antigen comes, it binds to the receptor and gets internalized into a phagosome phagosome will have lysosome fuses ultimately, foreign antigen undergoes digestion and exocytosis to get rid of cellular waste -***NEUTROPHILS have a variety of different cell surface receptors that recognize antigens -internalized by phagocytosis, lysosomes come, resulting waste undergoes exocytosis

the cervix is made up of __ cells

higher up it is made of reserve cells then changes to columnar, then to squamous, then to basal cells

what can increase your risk of breast cancer?

family history increases all of these -Mildly increased (1.5 - 2x) Moderate to florid hyperplasia (without atypia) Ductal papillomatosis Sclerosing adenosis -Moderately increased (5x): ADH/ALH (bilateral) -Significantly increased (8 -10x) LCIS (bilateral) DCIS (ipsilateral)

what are the two types of leukocytes?

granulocytes and agranulocytes: granulocytes: basophil, eosinophil, neutrophil agranulocytes: lymphocytes, monocytes

what is the composition of blood?

plasma erythrocytes (red blood cells) buffy coat

the fluid of the blood is called:

plasma it occupies ~55% of total blood volume, it is the liquid ECM

what is in the buffy coat?

platelets (120-300 thousand/cubic mm) 2 types of leukocytes WBC (5-10 thousand/cubic mm) 1.granulocytes: neutrophils PMNs (60-70%), eosinophils(2-4%) basophils(0.5-1%) 2.agranulocytes: monocytes(3-8%) lymocytes(20-25%)

findings of primary vs. secondary hemostasis:

primary hemostasis: platelet or vessel disorders - petchiae, bleeding from superficial cuts, most inherited disorders of women, mucosal bleeding secondary hemostasis: coagulation disorders deep hematomas, hemarthrosis, delayed bleeding, most inherited disorders of men, minimal mucosal bleeding

what is the proposed pathogenesis of cervical neoplasia?

sexual activty-> HPV exposure-> cervical transformation zone can go either to: squamous intraepithelial differentation->squmous intraepithlial lesion= which leads to low grade and high grade HPV risk -> invasive squamous cell carcinoma or to endocervical columnar differentiation-> glandular intraepithelial lesions->invasive adenocarcinoma

the distal urethra is lined with ___

squamous epithelium

the thymic cortex contains densely packed developing___

t-cells (thymocytes) Macrophages -phagocytose T-cells that do not fulfill thymic education requirements

what is the origin of platelets?

the originate from megakaryocytes

what is a von Willebrand factor deficiency ?

vWF Deficiency (platelet adhesion) -Very common- can be quantitative and/or qualitative defects Many type- I (milld quantitative), IIA, IIB, IIM, IIN (all qualitative), III (severe quantitative). -Varies in severity from asymptomatic to severe bleeding. -Normal and abnormal values vary according to blood type -Laboratory test for vWF quantity and function

what is non-neoplastic cellular growth in the vagina?

vaginitis Infectious vaginitis: Candida albicans Trichomonas vaginalis Non-Infectious vaginitis: Atrophic

what are the 3 types of ectopic pregnancies?

tubular pregnancy cornual pregnancy ovarian pregancy

what are the lymph nodes?

vascular system: -Afferent artery -Medullary arterioles -Cortical arterioles -High endothelial venules -Medullary venules -Efferent vein regions: Capsule, trabeculea, reticular tissue: connective tissue Cortex: aggregates of lymphocytes - lymphatic nodules, an active nodule has germinal center (GC) Paracortex: aggregation of T cells Medulla: medullary cords separated by medullary lymphatic sinuses

what is the composition of plasma?

water=92% by weight proteins=7%by weight albumins=58% globluins=37% fibrinogen=4% regulatory proteins=1% other solutes=1% by weight electrolytes, nutrients, respiratory gasses, waste products