UNIT 4 PATHO 545

CV-BETA 1-2-3 MEDICATION know beta 1, 2, 3 receptors of the heart and how medications affect each.

*(beta 1,2,3)* Stimulation of both the β1 and β2: Increases the heart rate (chronotropy) and force of the myocardial contraction (inotropy) If the heart rate is affected, then the effect is called chronotropy. Negative chronotropy: Decreases heart rate Positive chronotropy: Increases heart rate If the heart contraction is affected, then the effect is called inotropy. Negative inotropy: Decreases force of contraction Positive inotropy: Increases force of contrac Overall β1 and β2 stimulation Heart pumps more blood. β2 stimulation increases coronary blood flow. β3 receptors Decrease myocardial contractility (negative inotropic effect). May provide a "safety mechanism" to prevent an overstimulation of the heart by the sympathetic nervous system. *Medications affects on beta 1,2,3* Activation of the beta1 receptor leads to increases in contractile force and heart rate. activation of the beta 2 receptor inhibit calcium pathways in smooth muscle leading to relaxation. Activation of the β3 receptors induces the metabolism of lipids

RBC-SICKLE-CELL-ACUTE CHEST SYNDROME sickle-cell patho, inherited pattern, manifestation, dx, tx complications

*Acute chest syndrome* Sickled red blood cells attach to the endothelium of the injured, under ventilated, and inflamed lung and fail to be reoxygenated. Other form Sickle cell-Hb C disease: Is usually milder than sickle cell anemia.

RBC-SICKLE-CELL-APLASTIC CRISIS sickle-cell patho, inherited pattern, manifestation, dx, tx complications

*Aplastic crisis* - Transient cessation in red blood cell production occurs as a result of viral infection. - Sequestration crisis - Large amounts of blood pool in the liver and spleen - Hyperhemolytic crisis - Rate of red blood cell destruction is accelerated.

WLH-MONO-AKA EBSTEIN BARR VIRUS What is mononucleosis.

*Clinical manifestations* Malaise, arthralgia Classic triad of symptoms: Fever, pharyngitis, and lymphadenopathy of the cervical lymph nodes *Diagnostic test* Mono-spot qualitative test for heterophilic antibodies *Treatment* Rest and alleviation of symptoms with analgesics and antipyretics and penicillin or erythromycin Ibuprofen, not aspirin, for children and adolescents because of reported incidence of Reyes syndrome *what are the complications of mononucleosis* complications are rare may develop enlargement of spleen and liver. resulting in rupture of spleen. other fatalities are r/t hepatic failure extensive bacterial infection or viral myocarditis.

WLH- HIT

*Heparin Induced Thrombocytopenia (HIT)* immune-mediated peripheral consumption autoAb against platelet factor 4 & heparin *causes* thrombocytopenia due to rapid clearance of Ab-coated platelets THROMBOSIS is the problem (arterial or venous), not bleeding treat with coumadin for 3 months for prevention of thrombosis *clinical:* platelet counts decline 7-14 days after initial heparin exposure (can take as little as 24 hours if they've had previous exposure) *Dx:* ELISA assays for Ab to platelet factor 4 often gives false positives, but it's OK HEPARIN + DRAMATICALLY REDUCED PLATELETS = HIT UNTIL PROVEN OTHERWISE *TREATMENT:* remove hepain; give other anticoagulant if needed

RBC-ANEMIA-HEMOSTASIS REGULATION what regulates hemostasis? what VIT are needed for normal clotting factor synthesis?

*Major regulatory factors* - Endothelium prevents the formation of spontaneous -clots in normal vessels by several anticoagulant mechanisms. - Production of nitric oxide (NO) and prostacyclin I2 -(PGI2), thrombin inhibitors (antithrombin III), tissue -factor inhibitors (tissue factor pathway inhibitors), and -degrading activated clotting factors (thrombomodulin-protein C). *anticoagulant factors* - Antithrombin III --- Protease inhibitor: Inhibits thrombin and other clotting factors. - Tissue factor pathway inhibitor --- Reversibly inhibits factor Xa. - Proteins C and S --- Thrombomodulin system --- Degrades factors Va and VIIIa *lysis of blood clots* - limits the size of the clot and remove the clot after bleeding has ceased and repair has begun. *fibrinolytic system* - plasminogen and plasmin --- plasmin (fibrinase or fibrinolysin) degrades the clot. - tissue plasminogen activator (t-PA) for intravascular clot lysis - urokinase-like plasminogen activator (u-PA) for extravascular clot lysis. --- activates plasminogen to plasmin. - fibrin degradation products. --- d-dimers *VIT-REQUIREMENTS* VIT-K

HEMO-ANEMIA-MACROCYTIC NORMOCHROMIC-PERNICIOUS ANEMIA

*Pernicious anemia* - is the most common macrocytic anemia - is caused by a VIT B12 deficiency - lacks intrinsic factor from the gastric parietal cells. --- required for vit b12 absorption - may be a congenital or autoimmune disorder -- autoantibodies against intrinsic factor - conditions that increase risk -- past infection w/H Pylori -- gastrectomy -- proton-pump inhibitors *clinical manifestations* - weakness fatigue - paresthesia of the feet and fingers difficulty walking - sore tongue that is smooth and beefy red secondary to atrophic glossitis - lemon yellow skin as a result of a combination of pallor and icterus. - neurologic symptoms from nerve demyelination --- not reversible with treatment. - is often unrecognizable in older adults because of its subtle slow onset and presentation., *evaluation* - methylmalonic acid and homocysteine levels are elevated early in the disease. - gastric biopsy is diagnostic. *treatment* - parenteral or high doses of Vit B 12 are administered - left untreated death will result - lifelong tx required.

CVS-HYPERTENSION-RAAS know htn patho, etiology, RAAS system effect, clinical manifestations, dx, tx and complications/effects on other organs.

*RAAS* is associated with systolic heart failure. A. Activation of the RAAS cause not only increases in preload and afterload but also direct toxicity to the myocardium ANGiotensin II mediates remodeling of the ventricular wall, contributing to sarcomere death, loss of the normal collagen matrix and interstitial fibrosis. this leads to decreased contractility, changes in myocardial compliance and ventricular dilation. B. aldosterone no only causes salt and water retention by the kidney but also contributes to myocardial fibrosis, autonomic dysfunction, and dysrhythmias. it also has been implicated in endothelial dysfunction and prothrombotic effects.

HEMO-ANEMIA know all the anemias patho, etiology, clinical manifestations, dx, tx, compensation of the body and complications

*anemia* - is a reduction in the total number of erythrocytes in the circulating blood or in the quality or quantity of hemoglobin. -- impaired erythrocyte production -- acute or chronic blood loss -- increased erythrocyte destruction -- combination of the above *classification* - etiologic factor - size -- identified by terms that end in cytic -- macrocytic (large) , microcytic (small), normocytic (normal) -hemoglobin content -- identified by terms that end in chromic -- normochromic, hypochromic *anisocytosis* - RBC's are present in various sizes *poikilocytosis* - RBC are present in various shapes. *clinical manifestation* - reduced oxygen-carrying capacity: hypoxia - syncope, angina, compensatory tachycardian, and organ dysfunction - classic anemia symptoms --- fatigue, weakness, dyspena, elevated heart rate, and pallor *treatment* - transfusion, dietary correction, and admin of supplemental vitamins or iron - correction of the underling condition.

WLH-LYMPHOMA

*describe malignant lymphomas* Make up a diverse group of neoplasms that develop from the proliferation of malignant lymphocytes in the lymphoid system. *Primary lymphoid tissue* Thymus, bone marrow *Secondary lymphoid tissue* Lymph nodes, spleen, tonsils, intestinal lymphoid tissue *Two major categories* --Hodgkin lymphoma Linked to EBV ---Non-Hodgkin lymphoma Result from genetic mutations or a viral infection.

HEMO-ANEMIA-MACROCYTIC NORMOCHROMIC

*macrocytic-normochromic anemias* - aka megaloblastic anemias - RBC's are unusually large - deoxyribonucleic acid (DNA) synthesis is defective --- due to deficiencies in vitamin B12 or folate ----- co enzyme for nuclear maturation and the DNA synthesis pathway. - RNA processes occur at a normal rate -- results in unequal growth of the nucleus and cytplasm

RBC-ANEMIA-GRANULOCYTES know the function of all granulocytes.

*what are granulocytes* - membrane bound granules are in the cytoplasm - granules containing enzymes are capable of - destroying microorganisms - catabolize debris is ingested during phagocytosis - are involved in inflammatory and immune functions - are capable of amoeboid movement (diapedesis-- - movement to help granulocytes migrate through vessel - walls and then to sites where their action is needed). - aka phagocytes - contain many granules in cytoplasm - include neutrophils, basophils, and eosinophils. *neutrophils=do phagocytosis early in inflammation ;kill bacteria* - what are neutrophils - are the most numerous granulocyte (55%) - defend against infections - are referred to as polymorphonuclear neutrophils (PMNs) - serve as phagocytes in early inflammation - ingest and destroy microorganisms and debris and - then die in 1 or 2 days. - immature form: bands or stabs - mature form: segmented *basophils* - what are basophils - basophils make up < 1% of granulocytes - contain histamine - increase at the site of allergic inflammatory reactions - and parasitic infections, particularly ectoparasites (ticks) - secrete inflammatory mediators (histamine, -chemotactic factors for eosinophils and neutrophils) - contribute to the local inflammatory response. *eosinophils=worms and wheezes* - what are eosinophils - eosinophils make up 1-4% of the granulocytes. - are capable of amoeboid movement and phagocytosis - ingest antigen-antibody complexes and viruses - release cytokines and leukotrienes that augment the - inflammatory response - increase in type 1 hypersensitivity allergic reactions - and asthma - increase and attack parasitic infections. *mast cells* - are highly similar to basophils -are the central cell in inflammation - are found in vascularized connective tissue - activation and degranulation affect body cells --- increased permeability of blood vessels and smooth muscle contraction.

CV-- CHILDREN-HEART DISEASE-OBSTRUCTIVE DEFECTS know heart disease in children, what kind of murmur will you hear and where, how do children compensate for heart defects.

*pulmonary stenosis* - Narrowing of the pulmonary outflow tract - abnormal thickening of the valve leaflets - narrowing of the valve - pulmonary atresia :severe forms *clinical manifestations* -- often asymptomatic -- exertional dyspnea, murmur, fatigue *treatment* -- mild: not treated, closely observed -- severe: balloon angioplasty; pulmonary valvotomy *hypoplastic left heart syndrome* - left-sided cardiac structures develop abnormally -- obstruction to blood flow from the left ventricular outflow tract - left ventricle, aorta, and aortic arch are underdeveloped; mitral atresia or stenosis is observed *clinical manifestation* - as the ductus closes, systemic perfusion is decreased resulting in hypoxemia, acidosis, and shock *treatment* - prostaglandin administration - correction of acidosis - inotropic support for adequate cardiac output - ventilatory manipulation - surgical intervention includes three stages --- norwood procedure: atrial septectomy, pulmonary- to-systemic artery shunt, permanent communication between the right ventricle and aorta, and patching the hypoplastic aorta --- glenn procedure: performed between 2 and 9 months. the superior vena cava is joined to the pulmonary artery, and the take down to the shunt to the lungs --- fontan procedure: performed between 2 and 4 years of age; separates the systemic from the pulmonary circulation --- cardiac transplantation

CV-CIRCULATION know the heart circulation oxygenated deoxygenated blood changers valves cardiac cycle pressures

*right heart function* ˜Pumps blood through the lungs (pulmonary circulation). ˜Delivers blood to the lungs for oxygenation. ˜Is a low pressure system. *left heart function* Pumps oxygenated blood through the systemic circulation. Delivers metabolic waste products to the lungs, kidneys, and liver. Is a high pressure system. *what are arteries* Carry blood away from the heart. *what are capillaries* Exchange fluids between the blood and interstitial space *what are veins* carry blood to the heart *what is the 3 parts of the heart wall.* Epicardium: Outer smooth layer Myocardium: Thickest layer of cardiac muscle Endocardium: Innermost layer *what is the pericardium* Double-walled membranous sac Parietal: Surface layer Visceral: Inner layer; also called epicardium *what is the pericardial cavity* Space between the parietal and visceral layers Contains pericardial fluid (20 ml) *what are the chambers of the heart.* Right atrium Left atrium Atria are separated by the interatrial septum Right ventricle Left ventricle Are separated by the interventricular septum Thickness of each chamber depends on the pressure or resistance it must overcome to eject blood *what are the atrioventricular valves (AVs)* One-way flow of blood from the atria to the ventricles Tricuspid valve: Three leaflets or cusps Bicuspid (mitral) valve: Two leaflets or cusps *what are the semilunar valves* One-way flow from the ventricles to either the pulmonary artery or to the aorta Pulmonic semilunar valve Aortic semilunar valve *what are the great vessels* the superior and inferior venae cavae right and left pulmonary arteries. pulmonary veins aorta *what are the superior and inferior venae cavae* Bring deoxygenated blood from the systemic circulation to the right atria. *what are the right and left pulmonary arteries* Transport deoxygenated blood from the right heart to the right and left lungs. Branch into pulmonary capillaries *what are the pulmonary veins* carry oxygenated blood from the lungs to the left side of the heart. *what is the aorta* Delivers oxygenated blood to systemic vessels that supply the body

CV-CHILDREN-CONGENITAL DEFECTS-SLIDE 2 what causes mixed pulmonary blood flow.

*truncus arteriosus* - is the failure of the embryonic artery to divide into the pulmonary artery and aorta. - trunk straddles an always present VSD - types I-IV --- I= most common (80%); the main pulmonary artery arises from the truncus --- II= 20%; pulmonary arteries arise from the posterior aspect of the truncus --- III= 10%; pulmonary arteries arise from the lateral aspect of the truncus --- IV= pseudotruncus; is a severe form of tetralogy of fallot with the bronchial arteries arising from the descending aorta to supply the lung *Clinical manifestation* - cyanosis; mild to moderate cyanosis that worsens with activity *treatment* - modified rastelli procedure involving VSD patch closure to divert the blood flow from the left ventricle outflow tract into the truncus - correct pulmonary arteries. *kawasaki disease* - aka mucocutaneous lymph node syndrome - is an acute, self limiting systemic vasculitis that may result in cardiac sequelae - approximately 80% of cases occur in children <5 *cause* - unknown - theories: an immunologic response to an infectious toxic or antigenic substance (superantigen) *clinical manifestation* -*must have 5 of the 6 symptoms* -- fever for 5 days or longer unresponsive to antibiotics -- bilateral conjunctivitis w/out exudation -- erythema of oral mucosa (strawberry tongue) -- changes in extremities such as peripheral edema and erythema with desquamation of palms and soles -- polymorphous rash -- cervical lymphadenopathy *treatment* - aspirin and IV admin of immunoglobulin during the the acute phase. *SYSTEMIC HYPERTENSION* - hypertension in children differs from adults -- children often have underlying renal disease or coarctation of the aorta. -- cause of hypertension in children is almost always found -- children with htn are commonly asymptomatic *clinical manifestation* - systolic and diastolic b/p levels are greater the the 95th percentile for ages and gender on 2 out of 3 occasions *treatment* - appropriate diet - regular exercise - avoid smoking, drugs.

CV-CORONARY BLOOD FLOW-OCCLUSION coronary artery blood flow and effects of occlusions-which part of the heart is affected by occlusion in each coronary artery

*what are collateral arteries. * Are connections, or anastomoses, between the branches of the coronary circulation. Protects the heart from ischemia. Are formed by arteriogenesis or angiogenesis *what are coronary capillaries. * Where the exchange of oxygen and other nutrients takes place *what are the coronary veins* Coronary sinus Great cardiac vein Posterior vein of the left ventricle *how do blood and fluids pass through the coronary vessels. * Oxygenated blood enters the coronary arteries through openings in the semilunar valves at the entrance to the aorta. Deoxygenated blood from the coronary veins enters the right atrium through the coronary sinus. Coronary lymphatic vessels drain fluid to the paratracheal lymph nodes *what type of MI is caused by occlusion of the right coronary artery. * posterior inferior MI *what type of MI is caused by an occlusion of the left coronary artery* massive anterolateral MI *what type of MI is caused by occlusion of the left anterior descending artery* anteroseptal MI *what type of MI is caused by occlusion of the left circumflex coronary artery* lateral MI.

WLH-LEUKEMIAS What are the leukemias patho, clinical manifestation, diagnostic, tx, and complications.

*what are leukemias* Are malignant disorders of the blood and blood-forming organs. Exhibit uncontrolled proliferation of malignant leukocytes. Overcrowding of bone marrow Decreased production and function of normal hematopoietic cells Classification Predominant cell of origin: Myeloid or lymphoid Rate of progression: Acute or chronic *what are the clinical manifestations of leukemia* Clinical manifestations Fatigue caused by anemia Bleeding resulting from thrombocytopenia (reduced numbers of circulating platelets) Fever caused by infection Anorexia, weight loss, diminished sensitivity to sour and sweet tastes, wasting away of muscle, and difficulty swallowing Central nervous system (CNS) involvement *what are the test and treatments for leukemia* *Tests* Peripheral blood smear; bone marrow tests *Treatment* Chemotherapy Supportive measures Blood transfusions, antibiotics, antifungals, antivirals Allopurinol: Prevents the production of uric acid (which is elevated from cellular death because of treatment) Stem cell transplantation Bone marrow transplant *what are the complications of leukemias* Anemia Treatment: Blood products Neutropenia Treatment: Granulocyte colony-stimulated factor (G-CSF) or granulocyte-macrophage colony stimulating factor (GM-CSF) Low WBC count Treatment: Colony-stimulating factors to prevent infections

WLH-LEUKEMIA-RISK FACTORS

*what are risk factors of leukemias* Cigarette smoking, exposure to benzene, and ionizing radiation Human immunodeficiency virus (HIV), hepatitis C, human T-lymphotropic virus type 1 (HTLV-1) Drugs that cause bone marrow depression

WLH-G-6-PD path, inherited pattern, clinical manifestations, dx, tx, and complications.

*what is Glucose-6-phosphate dehydrogenase deficiency (G6PD)* Is an inherited, X-linked, recessive disorder. G6PD: Enzyme helps erythrocytes maintain metabolic processes, despite injurious conditions. Deficiency shortens red blood cell lifespan. Is asymptomatic unless stressors are present. Without G6PD, oxidative stressors damage hemoglobin and the plasma membranes of erythrocytes (Heinz bodies). *what is the manifestation treatment for G6PD* Clinical manifestations Icterus neonatorum Acute hemolytic anemia Pallor, icterus, dark urine, back pain Between hemolytic episodes: No anemia; erythrocyte survival is normal *Treatment* Prevention: High-risk group tested For hemolysis: Blood transfusions and oral iron therapy Spontaneous recovery: Generally follows treatment

WLH-LEUKEMIA-ACUTE

*what is acute leukemia* Presence of undifferentiated or immature cells, usually blast cells Rapid onset with short survival

WLH-LEUKEMIAS-ALL What IS ALL leukemias patho, clinical manifestation, diagnostic, tx, and complications.

*what is acute lymphocytic leukemia* *Most common childhood leukemia* Greater than 30% lymphoblasts in bone marrow or blood Genetic anomaly: Philadelphia chromosome Reciprocal translocation results in abnormal chromosome. Occurs between chromosomes 9 and 22. Risk factors for children Prenatal x-ray exposure Postnatal exposure to high-dose radiation Viral infections with HTLV-1: Can cause a rare form of ALL and EBV Down syndrome

WLH-LEUKEMIAS-AML What IS AML leukemias patho, clinical manifestation, diagnostic, tx, and complications.

*what is acute myelogenous leukemia* Most common adult leukemia (mean age, 67 years) Down syndrome: Increases risk Results from Abnormal proliferation of myeloid precursor cells Decreased rate of apoptosis Arrest in cellular differentiation Mutation in the receptor tyrosine kinase FLT3 Risk factors Exposure to radiation, benzene, and chemotherapy Hereditary conditions

WLH-THALASSEMIAS- ALPHA TRAIT patho, inherited pattern clinical manifestations, dx, tx, complications

*what is alpha trait thalassemia * Symptom free, having mild microcytosis at most Alpha-thalassemia minor Clinical manifestations: Virtually identical to those of beta-thalassemia minor Alpha-thalassemia major Hydrops fetalis Fulminant intrauterine congestive heart failure Fetus has a grossly enlarged heart and liver. Diagnosis usually made postmortem

WLH-THALASSEMIAS-MAJOR patho, inherited pattern clinical manifestations, dx, tx, complications

*what is beta thalassemia major* severe anemia resulting in large cardiovascular burden may cause severe illness *what is the treatment for thalassemia major.* Treatment for thalassemia major Genetic counseling Blood transfusions Iron chelation therapy Splenectomy Bone marrow, cord blood, and stem cell transplantation: Is currently the only cure

WLH-THALASSEMIAS-MINOR patho, inherited pattern clinical manifestations, dx, tx, complications

*what is beta-thalassemia minor.* mild to moderate hypochromic-microcytic anemia, mild splenomegaly bronze coloring of the skin hyperplasia of the bone marrow usually asymptomatic.

WLH-LEUKEMIA-CHRONIC

*what is chronic leukemia* Predominant cell is mature but does not function normally Slow progression

RBC-ANEMIA-ERYTHROPOIESIS know erythropoiesis and the iron cycle? What are the nutritional requirements?

*what is erythropoiesis* - Development of RBCs - Erythrocytes derived from erythroblasts (normoblasts) - Maturation stimulated by erythropoietin - Secreted by kidneys in response to tissue hypoxia - Reticulocyte count - Immature erythrocytes - Index of erythropoietic activity - Indicates whether new RBCs are being produced - Sequence - In each step, the quantity of hemoglobin increases and the nucleus decreases in size *what is the regulation of erythropoiesis* - Numbers of circulating RBCs in healthy individuals - remain constant. - Peritubular cells of the kidney produce erythropoietin. - Hypoxia stimulates the production and release of - erythropoietin. - Erythropoietin causes an increase in RBC production - and release from the bone marrow. *what are the nutritional requirements for erythropoiesis* - Proteins (amino acids) - Structural support - Synthesis of hemoglobin - Vitamins - B12 - Synthesis of deoxyribonucleic acid (DNA), maturation - of erythrocytes, facilitator of folate metabolism - B6 and E and pantothenic acid - Heme synthesis - Folic acid (also called folate) - Synthesis of DNA and ribonucleic acid (RNA); - maturation of erythrocytes - C: Iron metabolism - Riboflavin: Oxidative reactions - Niacin: Needed for respiration in mature erythrocytes - Minerals - Iron: Hemoglobin synthesis - Copper: Required for mobilization of iron from tissues to plasma

RBC-ANEMIA-HEMO SYNTHESIS know the process of hemoglobin synthesis.

*what is hemoglobin synthesis* - Oxygen-carrying protein of the erythrocyte - Single erythrocyte, containing as many as 300 -hemoglobin molecules - Two pairs of polypeptide chains - Globulins - Four colorful iron-protoporphyrin complexes - Most common type of adult hemoglobin - Hemoglobin A: Two α-chains and two β-chains - Heme: Large, flat, iron-protoporphyrin disk that is synthesized in the mitochondria and can carry one molecule of oxygen

WLH- HEMOPHILIAS bleeding disorders patho, inheretid pattern, clincial manifestations, dx, teament, complications.

*what is heophilias* Serious bleeding disorders Hemophilia A (classic hemophilia): Factor VIII deficiency, X-linked Hemophilia B (Christmas disease): Factor IX deficiency Hemophilia C: Factor XI deficiency von Willebrand disease: Autosomal dominant trait of factor VIII deficiency Two primary defects: Point mutation and gene deletion *what is the clinical manifestations test and tx of hemophilias.* Clinical manifestations Hematoma formations Persistent bleeding from relatively minor traumatic lacerations *Tests* Phase III: Thrombin time Phase II: Prothrombin time Phase I: Activated partial thromboplastin time, prothrombin consumption time, thromboplastin generation test (most sensitive) *Treatment* Recombinant factor VIII Recombinant antihemolytic factor plasma; albumin-free method: For hemophilia A

WLH-HODGKIN'S LYMPHOMA *what is the clinical manifestations, tests, and treatment for hodgkin's, lymphoma.

*what is hodgkin lymphoma* B cell in the germinal center has unsuccessful immunoglobulin gene rearrangement; should undergo apoptosis but survives. Virus might be involved in the pathogenesis. Familial clustering suggests an unknown genetic mechanism. *Clinical manifestations* Enlarged painless neck lymph nodes Lymphadenopathy, causing pressure or obstruction Mediastinal mass Fever, weight loss, night sweats, pruritus, fatigue *Tests* Chest x-rays, lymphangiography, and biopsy (biopsy most indicative of Hodgkin lymphoma) *Treatment* Approximate cure rate: 75% Combined treatment with radiation therapy and chemotherapy High-dose chemotherapy with bone marrow or stem cell transplantation Monoclonal antibodies Nonmyeloablative allogeneic stem cell transplantation

CVS-HYPERTENSION know htn patho, etiology, RAAS system effect, clinical manifestations, dx, tx and complications/effects on other organs.

*what is hypertension* Prehypertension: 120 to 139 mm Hg systolic; 80 to 90 mm Hg diastolic Isolated systolic hypertension: Elevated systolic blood pressure accompanied by normal diastolic blood pressure Hypertension Consistent elevation of systemic arterial blood pressure Sustained elevation of 140 mm Hg systolic or higher OR 90 mm Hg diastolic or higher *what is primary and secondary hypertension* Primary (essential) hypertension No known cause; is 95% of those with hypertension. Secondary hypertension Is caused by altered hemodynamics from an underlying primary disease or drugs. Affects the entire cardiovascular system Systolic hypertension: Most significant factor in causing target organ damage Increases the risk for myocardial infarction (MI), kidney disease, and stroke *what is the patho of hypertension* genetic and environment lead to insulin resistance, dysfunction of the SNS, RAAS, adducin and natriuretic hormones, and inflammation which leads to vasoconstriction, and renal salt and water retention which leads to increased peripheral resistance, and increased blood volume which leads to sustained hypertension. *etiology of htn* Positive family history Advancing age Gender: Female >70 years of age; male <55 years of age Race: Black Sodium (Na+) intake Glucose intolerance (diabetes mellitus) Heavy alcohol use Obesity Cigarettes Potassium (K+), magnesium (Mg++), calcium (Ca++) Caused by increases in cardiac output or total peripheral resistance, or both cardiac output increased: Any condition that increases heart rate or stroke volume Peripheral resistance increased: Any factor that increases blood viscosity or reduces vessel diameter (vasoconstriction *what are the manifestations and dx of htn.* Clinical manifestations Early stages of hypertension have no clinical manifestations other than elevated blood pressure Called silent (lanthanic) disease *Diagnosis* Measurement of blood pressure on at least two separate occasions averaging two readings at least 2 minutes apart with the individual seated, the arm supported at heart level, after 5 minutes of rest, with no smoking or caffeine intake in the past 30 minutes *what is the treatment for htn* Reducing or eliminating risk factors Restricting sodium intake to 2.4 g/day, increasing potassium intake, restricting saturated fat intake, and adjusting calorie intake as required to maintain optimal weight Dietary approaches to stop hypertension (DASH) Cessation of smoking Exercise program that promotes endurance and relaxation *Pharmacologic therapies* Reduce risk of end-organ damage, and prevent major diseases such as myocardial ischemia and stroke. Thiazide diuretics and beta blockers Are effective for lowering blood pressure and preventing the cardiovascular complications of hypertension. Are associated with lipid disorders and glucose intolerance. Angiotensin-converting enzyme (ACE) inhibitors, angiotensin-receptor blockers (ARBs), or aldosterone antagonists Effective for those with heart failure, chronic kidney disease, after an MI, or a recurrent stroke Calcium channel blockers Combination of thiazide diuretics and other antihypertensives

CV-ENDOCARDITIS know patho, etiology, clinical manifestations, dx, tx, complications of endocarditis.

*what is infective endocarditis.* Inflammation of the endocardium from infectious agents *Most common:* Bacteria, especially streptococci, staphylococci, and enterococci Pathogenesis Endocardial damage Bloodborne microorganism adherence Formation of infective endocardial vegetations *what is the clinical manifestations* ˜Clinical manifestations Fever New or changed cardiac murmur Petechial lesions of the skin, conjunctiva, and oral mucosa Osler nodes: Painful erythematous nodules on the pads of the fingers and toes Janeway lesions: Non Painful hemorrhagic lesions on the palms and soles *Treatment* Was once a lethal disease, but morbidity and mortality have significantly diminished with antibiotics and improved diagnostic techniques. Antimicrobial therapy is generally administered for 4 to 6 weeks. Begin with intravenous and end with oral administration. May use a combination. Other drugs are administered to treat left heart failure, secondary to valvular dysfunction. Surgery to repair or replace the valve, as prescribed.

RBC-ANEMIA-METHEMOGLOBIN what is methemoglobins

*what is methemoglobin* is a form of the oxygen-carrying metalloprotein hemoglobin, in which the iron in the heme group is in the Fe3+ (ferric) state, not the Fe2+(ferrous) of normal hemoglobin .Methemoglobin cannot bind oxygen

WLH-MULTIPLE MYELOMA What are the MYELOMA patho, clinical manifestation, diagnostic, tx, and complications. electrolyte imbalances.

*what is multiple myeloma* Plasma cells proliferate in the bone marrow. Primary translocation involves immunoglobulin heavy chain on chromosome 14. Malignant plasma cells produce abnormally large amounts of one class of immunoglobulin. M protein: Abnormal antibody molecule Unattached light chains of the immunoglobulins (Bence Jones proteins) can pass through the glomerulus and damage the renal tubular cells *what is the manifestations test, and tx of multiple myeloma* *Clinical manifestations* Hypercalcemia, renal failure Anemia Lytic lesions (round, "punched out" regions of bone) Skeletal pain Hyperviscosity syndrome Recurring infections due to loss of the humoral immune response *Tests* Radiographic and laboratory studies; bone marrow biopsy *Treatment* Prognosis poor Combinations of chemotherapy, radiation therapy, and plasmapheresis (exchange) and bone marrow transplantation High-dose chemotherapy, followed by blood-forming stem cell transplantation Tandem transplant, or thalidomide, or both (are showing promise) Bisphosphonates: To reduce skeletal damage Hydration and diuretics: To maintain a high urine output Antibiotics: To treat recurring infections *multiple myeloma is often accompanied with what electrolyte imbalance* hypercalcemia from infiltration of the bone by malignant plasma cells and stimulation of osteoclasts to re-absorb bone.

WLH-LEUKEMIA-PANCYTOPENIA

*what is pancytopenia* Reduction in all cellular components of the blood

WLH-THALASSEMIAS patho, inherited pattern clinical manifestations, dx, tx, complications

*what is thalassemias* Autosomal recessive disorders Slowed or defective synthesis of the globin chains of the hemoglobin molecule: Alpha or beta Major: Homozygous inheritance Minor: Heterozygous inheritance In alpha-thalassemia, the α-chains are affected; ß-chains are affected in beta-thalassemia. Beta-thalassemia minor Beta-thalassemia major: Cooley anemia; can be fatal Alpha trait: Single gene Alpha-thalassemia minor: Two genes Alpha-thalassemia major: Four genes; fatal Hemoglobin H disease: Three genes

CV-CIRCULATION-CARDIAC CYCLE know the heart circulation oxygenated deoxygenated blood changers valves cardiac cycle pressures

*what is the cardiac cycle.* Cardiac cycle One contraction and one relaxation Makes up one heartbeat Diastole Relaxation: Ventricles fill Systole Contraction: Blood leaves the ventricles *what is phase 1 of the cardiac cycle. * Phase 1: Atrial systole or ventricular diastole *what is phase 2 of the cardiac cycle* Phase 2: Isovolumetric ventricular systole *what is phase 3 of the cardiac cycle. * Phase 3: Ventricular ejection (semilunar valves open) *what is phase 4 of the cardiac cycle* Phase 4: Isovolumetric ventricular relaxation (aortic valve closes) *what is phase 5 of the cardiac cycle. * Phase 5: Passive ventricular filling (mitral and tricuspid valves open)

CV-EKG READING

*what is the conduction system of the heart* Sinoatrial (SA) node Pacemaker of the heart Intranodal pathways Atrioventricular (AV) node Bundle of His (AV bundle) Right and left bundle branches Purkinje fibers Ventricular myocardium Cardiac excitation Propagation of cardiac action potentials Depolarization: Activation Inside of the cell becomes less negatively charged. Repolarization: Deactivation Membrane potential Refractory period Heart muscles cannot contract. Ensures that diastole (relaxation) will occur. Completes the cardiac cycle. *what is the normal electrocardiogram ECG consist of. * Sum of all cardiac action potentials P wave: Atrial depolarization PR interval: Time from the onset of atrial activation to the onset of ventricular activation QRS complex: Sum of all ventricular depolarizations ST interval: Ventricular myocardium depolarized QT interval: "Electrical systole" of the ventricles Varies inversely with the heart rate.

RBC-ANEMIA know the function and life span of RBC's and platelets

*what is the function and life span of red blood cells.* - Have biconcavity and reversible deformity. - Biconcavity: Shape provides a surface area and - - --volume ratio that are optimal for gas diffusion and -deformity. - Reversible deformity: Enables the erythrocyte to assume a more compact torpedo-like shape, squeeze through the microcirculation, and return to normal. - Have a 120-day life cycle. *what is the function and life span of a platelet.* - Are irregularly-shaped cytoplasmic fragments. - Are formed by the fragmentation of megakaryocytes. - Are essential for blood coagulation and the control of bleeding. - Are incapable of mitotic division. - Granules are generally proinflammatory. - Normal count is 140,000 to 340,000 platelets/mm3. - Live for 8 to 10 days and then are removed by the spleen.

RBC-ANEMIA- HEMOSTASIS MECHANISM what is the function of the blood vessels? what is the function of platelets? what is the function of clotting factors?

*what is the mechanisms of hemostasis and the components* Hemostasis: Arrest of bleeding -Components -Vasculature (endothelial cells and subendothelial matrix) -Platelets -Blood proteins (clotting factors) *what is the sequence of hemostasis. Sequence* Vascular injury leads to vasoconstriction - Formation of a platelet plug - Tissue factor activates coagulation cascade - Formation of a blood clot (secondary hemostasis) - Clot retraction and clot dissolution (fibrinolysis *what is the function of the platelets in hemostasis* - Help regulate blood flow into a damaged site by inducing vasoconstriction. - Initiate platelet-to-platelet interactions, resulting in the formation of a platelet plug. - Activate the coagulation (or clotting) cascade to stabilize the platelet plug. - Initiate repair processes including clot retraction and clot dissolution (fibrinolysis). *platelet counts* -- Normal platelet count is 140,000 to 340,000/mm3. -- If the platelet count drops below 100,000/mm3, then -become thrombocytopenic (abnormally low numbers of platelets); prolongation of normal clotting may result. - If the platelet count falls below 20,000/mm3, then spontaneous bleeding may occur. - If platelet numbers are elevated (thrombocytosis), then the risk for spontaneous blood clots (thrombosis), stroke, or heart attack increases *what is the function of clotting factor in hemostasis.* -Fibrin production - Intrinsic pathway -- Is activated when the Hageman factor (factor XII) contacts subendothelial substances exposed by vascular injury. - Extrinsic pathway -- Is the most dominant. -- Is activated when the tissue factor (tissue thromboplastin) is released by damaged endothelial cells. - Both pathways lead to a common pathway -- Prothrombin to thrombin -- Fibrinogen to fibrin *platelet plug formation* *what is adhesion in platelet plug formation Adhesion* Mediated by the binding of the platelet surface receptor glycoprotein-Ib (GPIb) to von Willebrand factor (vWF) *what is activation in platelet plug formation Activation* Smooth spheres change to spiny projections and degranulation (called platelet-release reaction), resulting in the release of various potent biochemicals. *what is aggregation in platelet plug formation Aggregation* Is facilitated by fibrinogen bridges between receptors on the platelets. Clot retraction: Fibrin strands shorten and become denser and stronger to approximate the edges.

CV-CIRCULATION-PATH OF BLOOD FLOW know the heart circulation oxygenated deoxygenated blood changers valves cardiac cycle pressures

*what is the path of blood flow in the heart. * Deoxygenated (venous) blood from systemic circulation enters the right atrium through the superior and inferior venae cavae. From the atrium, the blood passes through the right AV (tricuspid) valve into the right ventricle. In the ventricle, the blood flows from the inflow tract to the outflow tract and then through the pulmonic semilunar valve (pulmonary valve) into the pulmonary artery, which delivers it to lungs for oxygenation Oxygenated blood from the lungs enters the left atrium through the four pulmonary veins (two from the left lung and two from the right).From the left atrium, the blood passes through the left AV valve (mitral valve) into the left ventricle.In the ventricle, the blood flows from the inflow tract to the outflow tract and then through the aortic semilunar valve (aortic valve) into the aorta, which delivers it to the entire body

RBC-ANEMIA-HEMATOPOIESIS know the process of hematopoiesis where does it occur in the fetus, neonate, child, and adult.

- Is the process of blood cell production in adult bone - marrow or in the liver and/or spleen of the fetus. - Humans need 100 billion new blood cells per day. - Two stages: -- Mitosis (proliferation) -- Maturation (differentiation) - Continues throughout life to replace blood cells that -grow old and die, are killed by disease, or are lost -through bleeding

HEMO-ANEMIA-MYELOPROLIFERATIVE- POLYCYTHEMIA

- Polycythemia -- overproduction of RBCs occurs - relative polycythemia -- is a result of dehydration -- fluid loss results in relative increases of RBCs counts and hemoglobin and hematocrit values -- resolves with fluid intake. *ABSOLUTE POLYCYTHEMIA* PRIMARY - abnormal regulation of the multipotent hematopoietic stem cells. - polycythemia vera SECONDARY - increase in erythropoietin as a normal response to chronic hypoxia - inappropriate response to erythropoietin secreting tumors - most common *POLYCYTHEMIA VERA* - chronic neoplastic, nonmalignant condition - overproduction of the RBCs(frequently with increased levels of WBCs[leukocytes] and platelets [thrombocytosis]) - splenomegaly - is an acquired mutation in Janus Kinase 2 (JAK2) - negates the self-regulatory activity of JAK2 that allows the erythropoietin receptor to be constitutively active, regardless of the level of erythropoietin. *CLINICAL MANIFESTATION* - spleen becomes enlarged, frequently with abdominal pain and discomfort - as the disease progresses, blood cellularity and viscosity increases - intense, painful itching intensified by heat or exposure to water (aquagenic pruritus) *TREATMENT* - phlebotomy: withdrawal of 300-500cc of blood at a time to reduce erythrocytosis and blood volume - low dose asa - interferon-a

RBC-SICKLE-CELL sickle-cell patho, inherited pattern, manifestation, dx, tx complications

- disorder caused by the presence of an abnormal hemoglobin (Hb S) - mutation causes valine to be replaced by glutamic acid - is autosomal recessive - deoxygenation and dehydration; red blood cells - solidify and stretch into an elongated sickle shape - sickle cell trait: child inherits Hb S from one parent and - normal hemoglobin (Hb A) from the other ; the - heterozygous carrier rarely has symptoms. - Extent, severity, and clinical manifestations depend on - the percentage of hemoglobin that is Hb S. - Sickling is an occasional, intermittent phenomenon. - Decreased oxygen tension (Po2) of the blood (e.g., -hypoxemia) - Increased hydrogen ion concentration in the blood =-(decreased pH) - Increased plasma osmolality, decreased plasma -volume, and low temperature - Polymerization: Sickled erythrocyte stiffens, changing from a flexible, beneficial cell to an inflexible one that starves and damages tissues. - what are the different manifestations of sickle cell. - Vasoocclusive crisis (thrombotic crisis) - Sickling is in microcirculation, extremely painful, and symmetric - Hands and feet exhibit painful swelling (hand-foot - syndrome). *what is the clinical manifestation test and tx of sickle cell* Clinical manifestations Infection: Most common cause of death Glomerular disease: Hyposthenuria—the inability of the tubules of the kidneys to concentrate urine, bed wetting, proteinuria Gallstones or cholecystitis *Treatment* Prevention of crises Avoid fever, infection, acidosis, dehydration, constricting clothes, and exposure to cold Immediate correction of acidosis and dehydration with appropriate intravenous fluids Routine childhood immunizations, annual influenza vaccine, pneumococcal and meningococcal vaccines Infections: Aggressive antibiotic therapy Oxygen: Not needed unless the individual is hypoxic Management of pain Genetic counseling and psychological support

RBC-ANEMIA-AGRANULOCYTOSIS know the function of all the agranulocytes

- lymphocytes (t and b-cells) and monocytes - relatively few granules are contained in cytoplasm - monocytes and macrophages: phagocytes - include lymphocytes: immunocytes. *lymphocytes* 36% of leukocytes are the major cells of the immune system are mature T, B, and plasma cells lifespan: days, months, or years depending on type. *B lymphocytes(plasma cells)=produce antibodies against specific antigens* *natural killers kill tumor cells and virally infected cells* - found mainly in the peripheral blood and spleen - do not have to be induced y antigens - produce cytokines involved in the immune response. *monocytes and macrophages-* - make up the mononuclear phagocyte system (MPS) -- are found in tissue and lymphoid organs -- provide the main line of defense against bacteria in the bloodstream. -- cleanse the blood by removing old, injured or dead blood cells. - *monocytes: are the precursor to macrophage and dendritic cells. * *macrophages=active phagocytosis as part of the mononuclear phagocyte system process and present antigens; participate in wound healing* - remove old and damaged cells and large molecules from circulation - are the major antigen processing and antigen presenting cells that initiate immune responses - initiate wound healing and tissue remodeling. *dendritic cells= process antigens and present them to the lymphocytes* - extend projections (dendrites) into the tissue, and take on a neuron like appearance. - are the major antigen processing and antigen presenting cells that initiate immune responses

WLH-HODGKINS-REED-STERNBERG CELLS what are reed-sternberg cells in the lymph nodes.

Are necessary for the diagnosis but not specific to Hodgkin lymphoma. Are derived from malignant B cells that usually become binucleate. Release cytokines. what are the two types of reed-sternberg cells. --Classic Hodgkin lymphoma --Nodular lymphocyte-predominant Hodgkin

CV-- CHILDREN-HEART DISEASE know heart disease in children, what kind of murmur will you hear and where, how do children compensate for heart defects.

CLASSIFICATIONS BASED ON BLOOD FLOW *lesions increasing pulmonary blood flow* -- defects that shunt from high pressure left side to low pressure right side with pulmonary congestion acyanosis *lesions decreasing pulmonary blood flow* -- generally complex with right-to-left shunt and cyanosis *obstructive lesions* -- right- or left-sided outflow tract obstructions that curtail or prohibit blood flow out of the heart; no shunting *mixing lesions* -- desaturated blood and saturated blood mix in the chambers or great arteries of the heart. HEART FAILURE - is a common condition associated with congenital birth defects - is also called CHF - Occurs when the heart is unable to maintain sufficient cardiac output to meet the metabolic demands of the body - neurohumoral and hemodynamic changes create abnormal ventricular wall stress and cause the myocardium to hypertrophy. *CLINICAL MANIFESTATIONS* - poor feeding, failure to thrive - dyspnea, tachypnea, diaphoresis, retractions, grunting, nasal flaring, wheezing, coughing - skin changes, such as pallor or mottling - hepatomegaly - pulmonary overcirculation; predominant cause associated with congenital defects. *TREATMENT* - decrease cardiac workload and increase the efficacy of heart function - administer diuretics, such as lasix - reduce afterload; admin. ACE inhibitors and beta blockers - supplement calorie intake. *HYPOXEMIA* Is a condition associated with congenital birth defects - heart defects that allow desaturated blood to enter the systemic system without passing through the lungs result in hypoxemia and cyanosis. -- hypoxemia: arterial oxygen tension is below normal and results in low oxygen arterial saturations and cellular function alteration -- cyanosis: blue discoloration of mucous membranes nail beds; is the result of deoxygenated hemoglobin *EISENMENGER SYNDROME* - pulmonary vascular resistance increases that or exceed or equal vascular resistance, resulting in a reversal of shunting

CV-CHILDREN-CONGENITAL DEFECTS what defects decrease pulmonary blood flow

DEFECTS DECREASING PULMONARY BLOOD FLOW *TETRALOGY OF FALLOT* - syndrome represented by four defects -- VSD -- overriding aorta straddles the VSD -- pulmonary valve stenosis -- right ventricle hypertrophy *clinical manifestation* - cyanosis and clubbing, feeding difficulty, squatting - hypercyanotic spell or a "tet spell" that generally occurs with crying and exertion *treatment* - most cases corrected surgically in early infancy before 1 year of age; blalock-taussig shunt, prosthetic graft, patch *TRICUSPID ATRESIA* - imperforate tricuspid valve - no communication between the right atrium and the right ventricle - additional defects -- septal defect -- hypoplastic or absent right ventricle -- enlarged mitral valve and left ventricle -- pulmonic stenosis *clinical manifestation* - central cyanosis and growth failure - exertional dyspnea, tachypnea, and hypoxemia *treatment* - prostaglandin administration - blalock taussig shunt - rashkind procedure (balloon atrial septostomy) - band - closure of septal defects

CV-CHILDREN-CONGENITAL DEFECTS defects increasing pulmonary blood flow

DEFECTS INCREASING PULMONARY BLOOD FLOW *Patent ductus arteriosus (PDA)* - failure of the ductus arteriosus to close -- normally closes with 15 hours to 2 wks after birth - PDA allows blood to shunt from the pulmonary artery to the aorta *clinical manifestation* - continuous machinery type murmur *treatment* - surgical closure involving ligation by incision, catheter or video-assisted thoracoscopy. *Atrial septal defect* - abnormal communication between the atria -- allows blood to be shunted from the left to right *clinical manifestation* - often asymptomatic; dx by murmur *three types of defect* - ostium primum - ostium secundum - sinus venosus *treatment* - surgical closure before school age results in better health - synthetic patch *ventricular septal defect (VSD)* - abnormal communication between ventricles -- shunting from the high pressure left side to the low pressure right side - common congenital heart lesion (25-33%) - pulmonary overcirculation accounts for symptoms associated with a large VSD TYPES OF VSD - perimembranous - muscular - supracristal - AV canal or inlet *clinical manifestation* - heart failure - poor weight gain - murmur and systolic thrill *treatment* - patch - right ventriculotomy *atrioventricular canal defect* - results from nonfusion of the endocardial cushion - abnormalities demonstrated in the atrial and ventricular septa and AV valves - complete, partial, and transitional AVC defects *Clinical manifestations* - murmur - heart failure *treatment* -complete repair 3 and 6 months of life

CV-- CHILDREN-HEART DISEASE-HYPOXEMIA/CYANOSIS know heart disease in children, what kind of murmur will you hear and where, how do children compensate for heart defects.

DEFECTS THAT CAUSE HYPOXEMIA AND CYANOSIS - lesions that cause obstruction and shunting from the right side of the heart to the left side of the heart as in the *tetralogy of fallot* - defects involving the mixing of saturated and unsaturated blood as in the univentricular heart - transposition of the great arteries. *CLINICAL MANIFESTATIONS* - mild hypoxemia -- cyanosis only occasionally when stressed - severe hypoxemia -- feeding intolerance, poor weight gain, tachypnea, and dyspnea -chronic hypoxemia -- small for their age, may display cognitive and motor skill delays -- polycythemia, shortness of breath with exertion, easily fatigued, and exercise intolerance. -- clubbing of the nail beds.

WLH-DIC know the patho, clinical manifestations, diagnostics, tx, and complications.

DIC Disseminated intravascular coagulation; acquired secondary coagulation disorder characterized by depletion of thrombocytes & coagulation factors what is disseminated intravascular coagulation (DIC) Complex, acquired disorder: Clotting and hemorrhage simultaneously occur Sepsis, cancer or acute leukemia, trauma, blood transfusion *Cause:* Variety of clinical conditions that release tissue factor Causes an increase in fibrin and thrombin activity in the blood. Produces augmented clot formation and accelerated fibrinolysis. *clinical manifestation* Characterized by a cycle of intravascular clotting, followed by active bleeding. Is caused by the initial consumption of coagulation factors and platelets. Results from abnormally widespread and ongoing activation of clotting. Hemorrhage: Is secondary to the abnormally high consumption of clotting factors and platelets. Deposition of fibrin clots in the circulation interferes with blood flow, causing widespread organ hypoperfusion. Clinical manifestations demonstrate wide variability. Bleeding from venipuncture sites Bleeding from arterial lines Bleeding from surgical wounds Purpura, petechiae, and hematomas Symmetric cyanosis of the fingers and toes *what is the treatment for DIC* Treatment Remove the stimulus Restore hemostasis Control thrombosis Maintain organ function Administer replacement therapy

HEMO-RAYNAUD PHENOMENON what is raynaud's phenomenon patho?

Episodic vasospasm (ischemia) in the arteries and arterioles of the fingers; less commonly in the toes *Clinical manifestations*' Changes in skin color and sensation caused by ischemia *Raynaud phenomenon* Secondary to other systemic diseases or conditions *Treatment:* Arm exercises and medications *Raynaud disease:* Primary vasospastic disorderof unknown origin *Treatment:* Avoidance of emotional stress and cold and cessation of cigarette smoking

WLH-THROMBOSIS-GENETICS

Hereditary thrombophilias --inherited condition increase risk for thrombosis -- most are autosomal dominant --aka primary thrombophilia --defect in proteins involved in hemostasis.

WLH- NON-HODGKIN'S BURKITT LYMPHOMA what is burkitt lymphoma, clinical manifestation test and tx.

Highly aggressive B-cell non-Hodgkin lymphoma Very fast-growing tumor of the jaw and facial bones (Africa); rare in the United States EBV in 90% of cases *clinical manifestations* Abdominal swelling for people affected in the United States *tests* Biopsy or bone marrow findings *Treatment* Radiotherapy and cyclophosphamide Adjuvant monoclonal antibody therapy with rituxim

WLH-KERNICTERUS

Highly aggressive B-cell non-Hodgkin lymphoma Very fast-growing tumor of the jaw and facial bones (Africa); rare in the United States EBV in 90% of cases clinical manifestations Abdominal swelling for people affected in the United States tests Biopsy or bone marrow findings Treatment Radiotherapy and cyclophosphamide Adjuvant monoclonal antibody therapy with rituxim

HEMO-ANEMIA-MYELOPROLIFERATIVE-IRON OVERLOAD

Iron overload --Primary versus secondary --Hereditary hemochromatosis ----Common inherited, autosomal recessive disorder of ----iron metabolism ----Characterized by increased gastrointestinal iron ---absorption with subsequent tissue iron deposition ----Excess iron deposited in the liver, pancreas, heart, joints, and endocrine gland, causing tissue damage --Hereditary hemochromatosis (cont'd) ----Fatigue, malaise ----Abdominal pain, arthralgias, and impotence ----Hepatomegaly, abnormal liver enzymes, bronzed -skin, diabetes, and cardiomegaly ----Treatment: Phlebotomy; refrain from taking iron and ----vitamin C supplements and consuming raw shellfish; ----alcohol use in moderation ----Family screening

WLH-LYMPHOBLASTIC LYMPHOMA complication of non-hodgkins what is lymphoblastic lymphoma how do you tx it.

Is a relatively rare variant of non-Hodgkin lymphoma (2% to 4%). Accounts for almost one third of cases in children and adolescents with a male predominance. More than 85% have T-cell origins. Clones of relatively immature T-cells become malignant in the thymus. *manifestation* First sign: Painless lymphadenopathy in the neck. *treatment* Treatment: Combined chemotherapy with multiple drugs.

HEMO-ANEMIA-VIT B-12 how is b-12 given

Is given as a Intramuscular injection

WLH NON-HODGKIN'S LYMPHOMA what is the clinical manifestations, tests, and tx for non-hodgkin lymphoma.

Is now called B cell neoplasms and includes T-cell and natural killer (NK) neoplasms. Is the generic term for a diverse group of lymphomas. Is linked to chromosome translocations. Clonal expansion of B cells (80% to 90%), T cells, and/or NK cells occurs. Changes in proto-oncogenes and tumor-suppressor genes contribute to cell immortality and thus an increase in malignant cells. *Clinical manifestations* Localized or generalized painless lymphadenopathy Nodal enlargement and transformation over months or years Retroperitoneal and abdominal masses with symptoms of abdominal fullness and back pain Ascites (fluid in the peritoneal cavity) and leg swelling *Tests* Biopsy (primary means of diagnosis) *Treatment* Survival: Extended periods but less than the survival rate for Hodgkin lymphoma Dependent on the type (B cell or T cell), tumor stage, histologic status (low, intermediate, high grade), symptoms, age, and any co-morbidities Chemotherapy or radiation Combination of chemotherapy and radiation Monoclonal antibody: Rituximab Radioimmunotherapy: Combination of radiation therapy with monoclonal antibody therapy

CV-CHILDREN-CONGENITAL DEFECTS what causes mixed pulmonary blood flow.

MIXED DEFECTS *Transposition of the great arteries* - aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle - results in two separate, parallel circuits. -- unoxygenated blood continuously circulates through the systemic circulation. -- oxygenated blood continuously circulates through the pulmonary system - extrauterine survival requires communication between the two circuits *clinical manifestation* - cyanosis may be mild shortly after birth and worsen during the first day. *treatment* - surgery to switch the arteries. *total anomalous pulmonary venous connection (TAPVC)* - pulmonary veins connect to the right side of the heart, directly or indirectly ,through one or more systemic veins that drain into the right atrium - nonobstructive versus obstructive *clinical manifestations* - cyanosis *treatment* - obstructed lesions are repaired at the time of dx - unobstructed lesions are generally repaired during infancy - surgery: anastomosis of the common pulmonary vein to the left atrium; closure of the atrial septal defect.

CV-- CHILDREN-HEART DISEASE-OBSTRUCTIVE DEFECTS know heart disease in children, what kind of murmur will you hear and where, how do children compensate for heart defects.

OBSTRUCTIVE DEFECTS *Coarctation of the aorta* - narrowing of the lumen of the aorta that impedes blood flow (8% to 10% of defects) - is almost always found in the juxtaductal position but it can occur anywhere between the origin of the aortic arch and the bifurcation of the aorta in the lower abdomen. *clinical manifestation* - newborns usually exhibit CHF -- once the ductus closes rapid deterioration occurs from hypotension, acidosis, and shock. - older children -- hypertension in the upper extremities -- decreased or absent pulses in lower extremities -- cool mottled skin -- leg cramps during exercise. *treatment* - prostaglandin administration - mechanical ventilation - inotropic support - surgery. *aortic stenosis* - narrowing of the aortic outflow tract (5% of defects) - caused by malformation of fusion of the cusps - causes an increased workload on the left ventricle *clinical manifestations* - often asymptomatic - signs of exercise intolerance in preadolescence - syncopal episode, epigastric pain, and exertional chest pain in more severe forms. *treatment* - balloon aortic valvuloplasty; aortic valvotomy.

HEMO-LYMPHO-

SEE SIGBIO HANDOUTS

HEMO-VARICOSE VEINS how do varicose veins develop? patho?

Vein in which blood has pooled -Usually in the saphenous vein -Distended, tortuous, and palpable veins *Cause:* - Trauma or gradual venous distention, rendering valves incompetent -Altered connective tissue proteins, increased -proteolytic enzyme activity, and decreased -transforming growth factor B in vein walls -Chronic venous insufficiency -Inadequate venous return over a long period as a result of varicose veins, valvular incompetence -Venous stasis ulcers *Treatment* -varicose veins and chronic venous insufficiency leg elevation, compression stockings, and physical exercise -Endovenous ablation (radiofrequency and laser) and ultrasound-guided foam sclerotherapy -Surgical ligation and vein stripping

CV-RHEUMATIC FEVER know patho, etiology, clinical manifestations, dx, tx, complications of rheumatic fever

acute rheumatic fever *What is the most common cause of mitral stenosis* *what is the clinical manifestation* Is a diffuse, inflammatory disease caused by a delayed immune response to infection by the group A beta-hemolytic streptococci. Is a febrile illness. Inflammation of the joints, skin, nervous system, and heart If left untreated, rheumatic fever causes rheumatic heart disease. (It may have a genetic component.) Abnormal immune response to the M proteins that cross react with normal tissues Fibrinoid necrotic deposits: Aschoff bodies *Clinical manifestations* Carditis: Murmur Polyarthritis: Large joints mainly affected Chorea: Sudden, aimless, irregular, involuntary movements Erythema marginatum: Truncal rash *Treatment:* 10-day regimen of antibiotics, NSAIDs, may need antibiotics for 5 years

HEMO-ALBUMIN- Know the consequences of low albumin

albumin molecules are large and do not diffuse freely through the vascular endothelium and thus they maintain the critical colloidal osmotic pressure that regulates the passage of water and solutes into the surrounding tissues. water and solute particles tend to diffuse out of the arterial portions of the capillaries because the blood pressure is greater in arterial than in venous blood vessels. water and solutes move from tissue cells into the venous portions of the capillaries where the pressures are reversed, oncotic pressure being greater than intravascular pressure or hydrostatic pressure.* in the case of decreased production. or excessive loss of albumin the reduced oncotic pressure leads to excessive movement of fluid and solutes into the tissue and decreased blood volume.*

QUESTION-HEMO-HEMOSTATISIS Von Willebrand factor is 1. essential for platelet activation 2. necessary for platelet adhesion 3. needed to stimulate platelet aggregation 4. required for hageman factor to degrade platelets.

answer rationale 2. necessary for platelet adhesion. platelet adhesion is mostly mediated by the binding of platelet surface receptor glycoprotein-lb (GPlb) (in complex with clotting factor IX and V) to van Willebrand factor (vWF) protein is found in the subendothelial matrix and is released by endothelial cells and platelets. wrong 1. platelet activation=reorganization of the platelet cytoskeleton 3. stimulate platelet aggregation=TXA2 and ADP which induce functional fibrinogen receptors 4. hageman factor to degrade platelets=intrinsic pathway for clotting is activated in plasma contacts negatively charged subendothelial substances exposed by vascular injury.

QUESTION-ERYTHROCYTE-ANEMIA Anemia of chronic disease is caused by. 1. immunoglobulin G (IgG) binding to erythrocytes at normal body temperatures. 2. autoantibodies against erythrocytes surface antigens 3. reduced response to erythropoietin 4. paroxysmal nocturnal hemoglobinuria.

answer/rational 3. reduced response to erythropoietin ACD results from a combination of 1) decreased erythrocyte life span 2) suppressed production of erythropoietin 3) ineffective bone marrow metabolism and iron sequestration in macrophages. wrong 1. warm autoimmune hemolytic anemia is caused by IgG that binds optimally to erythrocytes at normal bod temperature. 2. autoimmune hemolytic anemias are acquired disorders caused by autoantibodies against antigens normally on the surface of erythrocytes. 4. paroxysmal nocturnal hemoglobinuria= results from mutation in the X-linked gene for phosphatidylinositol glycan

QUESTION-ERYTHROCYTE-ANEMIA a person is admitted with an autoimmune disease directed against the hematopoietic stem cells. the nurse knows this will produce. 1. aplastic anemia 2. iron deficiency anemia 3. sideroblastic anemia 4. fanconi anemia

answer/rationale 1. aplastic anemia is the result of bone marrow suppression or failure caused by an autoimmune disease directed against the hematopoietic stem cells that produces pancytopenia. wrong 2. iron deficiency anemia can arise from one of two different etiologies or combination of both; inadequate dietary intake or excessive blood loss 3. sideroblastic anemias are a heterogeneous group of disorders characterized by anemia of varying severity caused by a defect in mitochondrial heme synthesis 4. fanconi anemia is a rare genetic anemia characterized by pancytopenia resulting from defects in DNA repair. this anemia develops early in life and is accompanied by multiple congenital anomalies.

QUESTION-HEMO-HEMATOPOIESIS Which physiological process occurs in hematopoiesis? 1. niches control the differentiation of hematopoietic progenitor cells 2. the spleen as the primary adult site uses splenic pulp to develop RBCs 3. peyer patches in the bone marrow stimulate the growth process 4. Mesenchymal stem cells differentiate into hematologic cells.

answer/rationale 1. niches control differentiation of hematopoietic progenitor cells. the hematologic compartment of the bone marrow consists of a variety of cellular microenvironments,m called niches that control differentiation of hematopoietic progenitor cells wrong 2. spleen=splenic pulp site of fetal hematopoiesis 3. peyer patches=found in the small intestine 4. mesenchymal stem cells=stromal cells that can differentiate into a variety of cells.

HEMO-HEMATOPOIESIS-PHYSIOLOGIC PROCESS-QUESTION which physiologic process occurs in hematopoiesis? 1. Niches control the differentiation of hematopoietic progenitor cells 2. The spleen as the primary adult site uses splenic pulp to develop RBCs 3. peyer patches in the bone marrow stimulate the growth process 4. mesenchymal stem cells differentiate into hematologic cells.,

answer/rationale 1. niches control the differentiation of hematopoietic progenitor cells. the hematologic compartment of the bone marrow consists of a variety of cellular microenvironments called niches that control differentiation of hematopoietic progenitor cells. wrong. 2. spleen=site of fetal hematopoiesis 3. peyer patches= 4. mesenchymal stem cells= differentiate into a variety of cells

QUESTION-HEMO-ERYTHROPOIESIS-NUTRITION A person has an inadequate intake of folic acid what will happen to this person RBC's 1. impaired iron metabolism 2. impaired DNA synthesis 3. impaired hemoglobin synthesis 4. impaired heme metabolism

answer/rationale 2. impaired DNA synthesis folate is the second most importanat vit for erythrocyte production and maturation folate is necessary for DNA synthesis being a componenet of three of the four DNA basis (thymine, adenine, and guanine,)and RNA synthesis. wrong 1. iron metabolims= VIT C 3. hemoglobin synthesis=IRON 4. heme metabolism= vit b6, E, pantothenic acid.

QUESTION-HEMO-COMPONENTS A person has an infection with early inflammation. which agranulocyte is the primary immunogenic WBC 1. neutrophil 2. natural killer cell 3. lymphocyte 4. eosinophil

answer/rationale 3. lymphocyte- lymphocytes which are agranulocytes constitute approximately 36% of the total leukocyte count and are the primary cells of the immune response neutrophils ,basophils and eosinophils are granulocytes that act as phagocytes. lymphocytes are the primary cells of the immune system. wrong 1. neutrophils=primary granulocyte chief phagocytes of early inflammation 2. natural killers=kill some types of tumor cell and some virus infected cells w/out being induced by exposure 4. eosinophils=seen in type I hypersensitivity allergy parasitic invasion and asthma

HEMO-INFECTION-QUES a person has an infection with early inflammation. Which agranulocyte is the primary immunogenic WBC? 1. neutorphil 2. natural killer cell 3. lymphocyte 4. eosinophil

answer/rationale 3. lymphocytes which are agranulocytes, constitute approximately 36% of the total leukocyte count and are the primary cells of the immune response neutrophils, basophils, and eosinophils are granulocytes that act as phagocytes. lymphocytes are the primary cells of the immune system. wrong 1. neutrophil- primary granulocyte and chief phagocytes of early inflammation 2. natural killer- kill some types of tumor cells.virus infected cells w/out being induced by previous exposure 4. eosinophils-granulocytes seen in increased concentration in type 1 hypersensitivity reaction allergy parasite invasion and asthma.

QUESTION-ERYTHROCYTE-ANEMIA A person with a gastrectomy is seen in the clinic for generalized weakness, fatigue, difficulty walking, and abdominal pain. The nurse suspects this individual has: 1. folate deficiency anemia 2. eryptosis anemia 3. polycythemia anemia 4. pernicious anemia

answer/rationale 4. pernicious anemia- pernicious anemia is caused by inadequate or absent production of intrinsic factor (IF) by gastric parietal cells. Complete or partial removal of the stomach (gastrectomy) causes (IF) deficiency. wrong 1. folate deficiency= produces scales and fissures of the lips and mouth, stomatitis, buccal and tongue ulcers, dysphagia, flatulence, and water diarrhea. 2. eryptosis-premature death of damaged erythrocytes, eryptosis, is a common mechanism of cellular loss in individuals with anemias secondary to deficiencies of iron, infections, chronic diseases and myelodysplastic syndrome 3. polycythemia=conditions in which erythrocyte numbers or volume is excessive.

QUESTION-HEMO-CHILD Which info is correct regarding infant or child's hematologic system. 1. blood cell counts decrease at birth from the loss of blood and then increase throughout childhood 2. immediately after birth of a full term neonate, the reticulocyte count decrases 3. platelets increase at birth and decrease to adult levels 4. polycythemia of the newborn occurs from hypoxic intrauterine environments.

answer/rationale 4. polycythemia of the newborn occurs from the hypoxic intrauterine environment the hypoxic intrauterine environment stimulates erythropoietin production in the fetus and accelerates fetal erythropoiesis, producing polycythemia (excessive proliferation of erythrocyte precursors) of the newborn) wrong. 1. blood cell count decreases=count tend to rise at birth 2. reticulocyte decrease= active fetal erythropoiesis results in large number of reticulocytes. 3. platelets increase=comparable with adults.

HEMO-ERYTHROPOIESIS-NUTRITIONAL REQ-QUESTION A person has an inadequate intake of folic acid (folate) what wil happen to this persons RBCs. 1. impaired iron metabolism 2. impaired dna synthesis 3. impaired hemoglobin synthesis 4. impaired heme metabolism

answer/ratonale 2. impaired DNA synthesis= folate is the second most important vitamin for erythrocyte production and maturation. folate

HEMO-CASE STUDY Joan riley age 56 came to her NP with fatigue pallor dyspnea on exertion, and palpitations. her laboratory report indicates that her hematocrit , hemoglobin, and reticulocyte count are low, that her MCV is high and that her MCH and MCHC are normal. Her dxs is pernicious anemia. A. why should the NP ask joan about her paresthesias and ataxia B. why did the NP prescribe Vit B12 by IM injection rather than orally C. What causes pernicious anemia D. What is the technical term that describes an anemia with high MCV and normal MCH.

answers/rationale A. Vit B12 deficiency causes neurological abnormalities such as paresthesias, ataxia, or muscle weakness. B. without the presence of intrinsic factor, vit B12 will not be absorbed effectively from the gastrointestinal tract. C. lack of intrinsic factor, a substance normally produced in the stomach that enables absorption of vit 12 D. Macrocytic normochromic anemia.

CV-HIV/AIDS What are the cardiac complications in patients HIV/AID

dilated cardiomyopathy, pericardial effusion, human immunodeficiency virus-associated pulmonary hypertension, endocarditis, thrombosis, embolism, vasculitis, coronary artery disease, aneurysm cardiac involvement in AIDS-related tumors.

WLH-HEMOLYTIC DISEASE OF THE NEWBORN

hemolytic disease of the newborn (HDN) - is also termed erythroblastosis fetalis -- as hemolysis progresses, the fetus becomes anemic; as a result, erythropoiesis accelerates and immature nucleated cells (erythroblasts) are released into the bloodstream, hence the name. -- is an alloimmune disease -- maternal blood and fetal blood are antigenically incompatible -- maternal antibody is directed against fetal antigens -- between 20% and 25% are ABO incompatible; less than 10% are Rh incompatible but is more severe. *clinical manifestation* -anemia -hyperbilirubinemia -icterus neonatorum (neonatal jaundice) - *kernicterus*; bilirubin deposited in the brain and can cause death *test* -coombs *treatment* -prevention Rh immune globulin (RhoGAM) -if not treated phototherapy exchange transfusion

RBC- ANEMIA- SPLEEN what is the function of the spleen what happens when pt has splenectomy.

is similar to the lymph node except that it is larger and filled with blood. serves as a reservoir (a holding place) for blood will, if removed or damaged, leave the body more susceptible to infections filters and purifies the blood and lymph fluid that flows through it Splenomegaly: May be classified as pathologic Hypersplenism: Overactive spleen Congestive splenomegaly: Occurs with hepatic cirrhosis Infiltrative splenomegaly: Engorgement by macrophages with indigestible materials from various "storage diseases" Manifestation: Anemia from red blood cell (RBC) destruction Treatment: Removal of the spleen *splenectomy* After having a splenectomy, the patient must always be extra careful about infections. The doctor is likely to have immunized the patient before surgery. Other likely recommendations include: Have a pneumonia vaccine about every five years. Have yearly flu shots. A child who has had his or her spleen removed may be put on antibiotics for two to several years after the surgery, possibly until adulthood. Contact your doctor immediately if you have a fever or other indications of infection. Avoid travel to places where you could contract malaria.

HEMO-LEUKOCYTES-ALLERGIC REACTION what happens to leukocytes during a type I allergic reaction?

mechanism: anaphylaxis. mediated release of IgE,-> mast cells basophils.

WLH-LEUKEMIAS-CLL What IS CLL leukemias patho, clinical manifestation, diagnostic, tx, and complications.

what is chronic lymphocytic leukemia Affects monoclonal B lymphocytes. Has a familial tendency. Is common in adults older than 50 years *what is the clinical manifestations of CLL* Advances slowly and insidiously. CLL Is asymptomatic at the time of diagnosis. Lymphadenopathy is the most common finding. Suppresses humoral immunity, and increases infection with encapsulated bacteria.

WLH-LEUKEMIAS-CML What IS CML leukemias patho, clinical manifestation, diagnostic, tx, and complications.

what is chronic myelogenous leukemia Is usually diagnosed in adults. Is a myeloproliferative disorder that also includes polycythemia vera, primary thrombocytosis, and idiopathic myelofibrosis. Philadelphia chromosome is often present and BCR-ABL1 causes initiation of CML 80. *what is the clinical manifestations of CML* Infections, fever, and weight loss Chronic phase Lasts 2 to 5 years Symptoms: May not be apparent Accelerated phase Lasts 6 to 18 months Primary symptoms develop: Splenomegaly Terminal blast phase "Blast crisis" Survival: Only 3 to 6 months *what are the test and tx for CML* ˜Tests Blood smear ˜Treatment Chlorambucil, administered with or without corticosteroids, on a daily or intermittent schedule Chemotherapy No cure for CML •Combined chemotherapy •Biologic response modifiers •Allogeneic stem cell transplantation