Chp 30- Hematologic Problems Terms
risk factors for DIC
*Acute DIC* Shock • Hemorrhagic • Cardiogenic • Anaphylactic Septicemia (bacterial, viral, fungal, parasitic) Hemolytic processes • Transfusion of mismatched blood • Acute hemolysis from infection or immunologic disorders Obstetric conditions • Abruptio placentae • Amniotic fluid embolism • Septic abortion • HELLP syndrome Malignancies • Acute leukemia • Metastatic solid tumors Tissue damage • Extensive burns and trauma • Heatstroke • Severe head injury • Transplant rejections • Postoperative damage, especially after extracorporeal membrane oxygenation • Fat and pulmonary emboli • Snakebites • Glomerulonephritis • Acute anoxia (e.g., after cardiac arrest) • Prosthetic devices • Fulminant hepatitis *Subacute DIC* Malignancy • Myeloproliferative/lymphoproliferative malignancies • Metastatic cancer Obstetric • Retained dead fetus *Chronic DIC* Liver disease Systemic lupus erythematosus Malignancy DIC, Disseminated intravascular coagulation; HELLP syndrome, a life-threatening liver disorder thought to be a type of severe preeclampsia, it is characterized by Hemolysis (destruction of red blood cells), Elevated Liver enzymes (which indicate liver damage), and Low Platelet count.
etiologic classification of anemia
*Decreased RBC Production* Decreased Hemoglobin Synthesis • Iron deficiency • Thalassemias (decreased globin synthesis) • Sideroblastic anemia (decreased porphyrin) Defective DNA Synthesis • Cobalamin (vitamin B12) deficiency • Folic acid deficiency Decreased Number of RBC Precursors • Aplastic anemia and inherited disorders (e.g., Fanconi syndrome) • Anemia of myeloproliferative diseases (e.g., leukemia) and myelodysplasia • Chronic diseases or disorders • Medications and chemicals (e.g., chemotherapy, lead) • Radiation *Blood Loss* Acute • Trauma • Blood vessel rupture • Splenic sequestration crisis Chronic • Gastritis • Menstrual flow • Hemorrhoids *Increased RBC Destruction (Hemolytic Anemias)* Hereditary (Intrinsic) • Abnormal hemoglobin (sickle cell disease) • Enzyme deficiency (G6PD) • Membrane abnormalities (paroxysmal nocturnal hemoglobinuria, hereditary spherocytosis) Acquired (Extrinsic) • Physical destruction • Prosthetic heart valves • Extracorporeal circulation • Disseminated intravascular coagulopathy (DIC) • Thrombotic thrombocytopenic purpura (TTP) • Antibodies against RBCs • Infectious agents (e.g., malaria) and toxins G6PD, Glucose-6-phosphate dehydrogenase.
nursing assessment of leukemia
*Subjective Data* Important Health Information Past health history: Exposure to chemical toxins (e.g., benzene, arsenic), radiation, or viruses (Epstein-Barr, HTLV-1); chromosome abnormalities (Down syndrome, Klinefelter syndrome, Fanconi syndrome), immunologic deficiencies; organ transplantation; frequent infections; bleeding tendencies Medications: Chemotherapy Surgery or other treatments: Radiation exposure; prior radiation and chemotherapy for cancer Functional Health Patterns Health perception-health management: Family history of leukemia; malaise Nutritional-metabolic: Mouth sores, weight loss; chills, night sweats; nausea, vomiting, anorexia, dysphagia, early satiety; easy bruising Elimination: Hematuria, decreased urine output; diarrhea, dark or bloody stools Activity-exercise: Fatigue with progressive weakness; dyspnea, epistaxis, cough Cognitive-perceptual: Headache; muscle cramps; sore throat; generalized sternal tenderness, bone, joint, abdominal pain; paresthesias, numbness, tingling, visual disturbances Sexuality-reproductive: Prolonged menses, menorrhagia, metrorrhagia, impotence *Objective Data* General Fever, generalized lymphadenopathy, lethargy Integumentary Pallor or jaundice; petechiae, ecchymoses, purpura, reddish brown to purple cutaneous infiltrates, macules, and papules Cardiovascular Tachycardia, systolic murmurs Gastrointestinal Gingival bleeding and hyperplasia; oral ulcerations, herpes and Candida infections; perirectal irritation and infection; hepatomegaly, splenomegaly Neurologic Seizures, disorientation, confusion, decreased coordination, cranial nerve palsies, papilledema Musculoskeletal Muscle wasting, bone pain, joint pain Possible Diagnostic Findings Low, normal, or high WBC count with shift to the left (↑ blast cells) and neutropenia; anemia, ↓ hematocrit and hemoglobin, thrombocytopenia, Philadelphia chromosome; hypercellular bone marrow aspirate or biopsy with myeloblasts, lymphoblasts, and markedly ↓ normal cells HTLV-1, Human T-cell leukemia virus, type 1.
nursing assessment of anemia cont.
*Subjective Data* Important Health Information Past health history: Recent blood loss or trauma; chronic liver, endocrine, or renal disease (including dialysis); GI disease (malabsorption syndrome, ulcers, gastritis, or hemorrhoids); inflammatory disorders (especially Crohn's disease); smoking, exposure to radiation or chemical toxins (arsenic, lead, benzenes, copper); infectious diseases (HIV) or recent travel with possible exposure to infection; angina, myocardial infarction; history of falling Medications: Use of vitamin and iron supplements; aspirin, anticoagulants, oral contraceptives, phenobarbital, penicillins, nonsteroidal antiinflammatory drugs, omeprazole, phenacetin, phenytoin (Dilantin), sulfonamides, herbal products Surgery or other treatments: Recent surgery, small bowel resection, gastrectomy, prosthetic heart valves, chemotherapy, radiation therapy Dietary history: General dietary patterns, consumption of alcohol Functional Health Patterns Health perception-health management: Family history of anemia; malaise Nutritional-metabolic: Nausea, vomiting, anorexia, weight loss; dysphagia, dyspepsia, heartburn; night sweats, cold intolerance Elimination: Hematuria, decreased urine output; diarrhea, constipation, flatulence, tarry stools, bloody stools Activity-exercise: Fatigue, muscle weakness and decreased strength; dyspnea, orthopnea, cough, hemoptysis; palpitations; shortness of breath with activity Cognitive-perceptual: Headache; abdominal, chest, and bone pain; painful tongue; paresthesias of feet and hands; pruritus; disturbances in vision, taste, or hearing; vertigo; hypersensitivity to cold; dizziness Sexuality-reproductive: Menorrhagia, metrorrhagia; recent or current pregnancy; male impotence *Objective Data* General Lethargy, apathy, general lymphadenopathy, fever Integumentary Pale skin and mucous membranes; blue, pale white, or icteric sclera; cheilitis (inflammation of the lips); poor skin turgor; brittle, spoon-shaped fingernails; jaundice; petechiae; ecchymoses; nose or gingival bleeding; poor healing; dry, brittle, thinning hair Respiratory Tachypnea Cardiovascular Tachycardia, systolic murmur, dysrhythmias; postural hypotension, widened pulse pressure, bruits (especially carotid); intermittent claudication, ankle edema Gastrointestinal Hepatosplenomegaly; glossitis; beefy, red tongue; stomatitis; abdominal distention; anorexia Neurologic Headache, roaring in the ears, confusion, impaired judgment, irritability, ataxia, unsteady gait, paralysis, loss of vibration sense Possible Diagnostic Findings ↓ RBCs, ↓ Hgb; ↓ Hct; ↑ or ↓ reticulocytes, ↑ or ↓ MCV; possible ↓ serum iron, ferritin, folate, or cobalamin (vitamin B12); heme (guaiac)-positive stools; ↓ serum erythropoietin level; ↑ or ↓ LDH, bilirubin, transferrin (Table 30-6) LDH, Lactate dehydrogenase; MCV, mean corpuscular volume.
nursing assessment of thrombocytopenia
*Subjective Data* Important Health Information Past health history: Recent hemorrhage, excessive bleeding, or viral illness; HIV infection; cancer (especially leukemia or lymphoma); aplastic anemia; systemic lupus erythematosus; cirrhosis; exposure to radiation or toxic chemicals; disseminated intravascular coagulation Medications: Many medications including chemotherapy agents, valproic acid (Depakote), furosemide (Lasix), gold, nonsteroidal antiinflammatory drugs, penicillin Functional Health Patterns Health perception-health management: Family history of bleeding problems; malaise Nutritional-metabolic: Bleeding gingiva; coffee-ground or bloody vomitus; easy bruising Elimination: Hematuria, dark or bloody stools Activity-exercise: Fatigue, weakness, fainting; epistaxis, hemoptysis; dyspnea Cognitive-perceptual: Pain and tenderness in bleeding areas (e.g., abdomen, head, extremities); headache Sexuality-reproductive: Menorrhagia, metrorrhagia *Objective Data* General Fever, lethargy Integumentary Petechiae, ecchymoses, purpura Gastrointestinal Splenomegaly, abdominal distention; guaiac-positive stools Possible Diagnostic Findings Platelet count <150,000/µL (150 × 109/L), prolonged bleeding time, ↓ hemoglobin and hematocrit; normal or ↑ megakaryocytes in bone marrow examination
acquired hemolytic anemia
- Normal red cells but unable to survive due to a "hostile environment", damage is caused by external factors - Attacked and destroyed by antibodies. - Destruction of red cells by mechanical trauma. - Passing through enlarged spleen. [splenomegaly] - In contact with some part of artificial heart valve.
aplastic crisis
A condition in which the body stops producing red blood cells; typically caused by infection. -seen in SCD pts
polycythemia
A disorder characterized by an abnormal increase in the number of red blood cells in the blood; circulation may be impaired as result of increased blood viscosity (hyperviscosity) & volume (hypervolemia); absolute increase in RBC mass -2 types 1) primary 2) secondary -manifestations: hypertension (caused by hypervolemia & hyperviscosity) *early s/sx: headache, vertigo, dizziness, tinnitus, visual disturbances, generalized pruritus *later s/sx: paresthesias/erythromelalgia, angina, HF, thrombophlebitis, plethora, hyperuricemia & possible gout (since when RBC destruction increases, so does uric acid) -labs/diagnosis: elevated hemoglobin & RBC, low to normal EPO level (except for secondary), elevated WBC, elevated platelets & platelet dysfunction, electated leukocyte, uric acid, and cobalamin levels, elevated histamine -nursing management: > reduce blood volume & viscosity > phlebotomy is mainstay of tx > avoid iron & hydrate > too much fluid may cause overload (furthering complications) & underhydrating may cause further blood viscosity) so assess I/O carefully > low dose aspirin may be used to prevent clotting
hemophilia
A hereditary disease where blood does not coagulate to stop bleeding, caused by a defective or deficient coagulation factor -2 major types 1) hemophilia A: classic & most common, factor VIII deficiency * though rare, there are cases of acquired hemophilia A 2) hemophilia B: christmas disease, factor IX deficiency
classification of non-hodgkin's lymphoma
B-Cell Lymphomas • Diffuse large B-cell lymphoma (DBLCL) • Follicular lymphoma • Marginal zone B-cell lymphoma (MALT) • Small lymphocytic lymphoma/chronic lymphocytic leukemia • Mantle cell lymphoma • Burkitt's lymphoma • Plasmablastic lymphoma • AIDS-related B-cell lymphomas T-Cell and NK-Cell Lymphomas • Lymphoblastic lymphoma • T-cell lymphoma • Mycosis fungoides and Sézary syndrome • Anaplastic large T-cell lymphoma • Natural killer cell lymphomas Posttransplant Lymphoproliferative Disorders (PTLD) MALT, Mucosa-associated lymphoid tissue. *This is only a partial list.
classification of megaloblastic anemia
Cobalamin (Vitamin B12) Deficiency • Dietary deficiency • Deficiency of gastric intrinsic factor • Pernicious anemia • Gastrectomy • Gastric bypass • Celiac disease • Helicobacter pylori • Intestinal malabsorption • Increased requirement (pregnancy) • Chronic alcoholism Folic Acid Deficiency • Dietary deficiency (e.g., leafy green vegetables, citrus fruits) • Malabsorption syndromes • Celiac disease • Crohn's disease • Small bowel resection • Drugs interfering with absorption or use of folic acid • Methotrexate • Antiseizure drugs (e.g., phenobarbital, phenytoin [Dilantin]) • Increased requirement (pregnancy) • Chronic alcoholism • Chronic hemodialysis (folic acid lost during dialysis) Drug-Induced Suppression of DNA Synthesis • Folate antagonists • Metabolic inhibitors • Alkylating agents Inborn Errors • Defective folate metabolism • Defective transport of cobalamin Erythroleukemia
interprofessional care of thrombocytopenia
Diagnostic Assessment • History and physical examination • Bone marrow aspiration and biopsy • CBC, including platelet count • Specific laboratory studies (Table 30-12) Management Immune Thrombocytopenic Purpura • Corticosteroids • IV immunoglobulin (IVIG) • Anti-Rho(D) • Rituximab (Rituxan) • Splenectomy • Romiplostim (Nplate) • Eltrombopag (Promacta) • Danazol • Immunosuppressives (e.g., cyclosporine, cyclophosphamide, azathioprine [Imuran], mycophenolate mofetil [CellCept]) • Platelet transfusions Thrombotic Thrombocytopenic Purpura • Identification and treatment of cause • Plasmapheresis (plasma exchange) • Corticosteroids • Rituximab (Rituxan) • Immunosuppressives (e.g., cyclophosphamide) • Chemotherapy (vincristine, vinblastine) • Splenectomy Heparin-Induced Thrombocytopenia • Direct thrombin inhibitor (lepirudin [Refludan], argatroban [Acova]) • Indirect thrombin inhibitor (fondaparinux [Arixtra]) • Warfarin (Coumadin) • Plasmapheresis (plasma exchange) • Thrombolytic agents Decreased Platelet Production • Identification and treatment or removal of cause • Corticosteroids • Platelet transfusions
interprofessional care of iron deficiency anemia
Diagnostic Assessment • History and physical examination • Hct and Hgb levels • RBC count, including morphology • Reticulocyte count • Serum iron • Serum ferritin • Serum transferrin • Total iron-binding capacity (TIBC) • Stool examination for occult blood Management • Identification and treatment of underlying cause • Drug therapy • Oral: Ferrous sulfate or ferrous gluconate • IM or IV: Iron dextran, sodium ferrous gluconate, iron sucrose • Nutritional therapy (Table 30-5) • Transfusion of packed RBCs
interprofessional care of neutropenia
Diagnostic Assessment • History and physical examination • Risk assessment for severity and duration of neutropenia • WBC count with differential count • WBC morphology • Hct and Hgb • Reticulocyte and platelet count • Bone marrow aspiration or biopsy • Cultures of nose, throat, sputum, urine, stool, obvious lesions, blood (as indicated) • Chest x-ray or other diagnostic tests Management • Identification and removal of cause of neutropenia (if possible) • Identification of site of infection (if present) and causative organism • Antimicrobial therapy (prophylactically or empiric)* • Blood cultures drawn STAT, immediately before antibiotics • Prophylactic hematopoietic growth factors (G-CSF, GM-CSF, pegfilgrastim [Neulasta]) after myelosuppressive chemotherapy • Strict hand washing • Patient hygiene (daily skin and frequent oral care; chlorhexidine bathing should be done in the inpatient setting if the patient has a central venous catheter) • Single-patient room, positive-pressure or high-efficiency particulate air (HEPA) filtration, depending on risk • Community isolation and home precautions (if outpatient) • Nutritional therapy (dietary instruction) on foods to avoid and safe food handling • Safe activity and ambulation to maintain physical and pulmonary function G-CSF, Granulocyte colony-stimulating factor; GM-CSF, granulocyte-macrophage colony-stimulating factor. *Prompt initiation of empiric broad-spectrum antibiotic therapy at any sign of infection, even a low-grade fever (100.4° F [38° C]), is essential.
causes of neutropenia
Drugs* • Antitumor antibiotics (daunorubicin [Cerubidine], doxorubicin [Adriamycin]) • Alkylating agents (nitrogen mustard, busulfan [Myleran]) • Antimetabolites (methotrexate, 6-mercaptopurine [6-MP], cytarabine) • Antiinflammatory drugs (phenylbutazone) • Cardiovascular drugs (captopril [Capoten], procainamide [Pronestyl]) • Diuretics (furosemide [Lasix]) • Miscellaneous (gold, penicillamine) • Antimicrobial agents (ganciclovir, penicillin G, trimethoprim/sulfamethoxazole [Bactrim]) Hematologic Disorders • Idiopathic neutropenia • Congenital (cyclic neutropenia) • Aplastic anemia • Fanconi syndrome • Leukemia • Myelodysplastic syndrome Autoimmune Disorders • Systemic lupus erythematosus • Felty syndrome • Rheumatoid arthritis Infections • Viral (e.g., hepatitis, influenza, HIV, measles) • Fulminant bacterial infection (e.g., typhoid fever, miliary tuberculosis) • Parasitic • Rickettsial Others • Severe sepsis • Bone marrow infiltration (e.g., carcinoma, tuberculosis, lymphoma) • Hypersplenism (e.g., portal hypertension, storage diseases [e.g., Gaucher disease]) • Nutritional deficiencies (cobalamin, folic acid) • Transfusion reaction • Hemodialysis *Not all inclusive.
hemochromatosis genetics
Genetic Basis • Autosomal recessive disorder • Caused by mutations in the HAMP, HFE, HFE2, SLC40A1, and TFR2 genes • These genes play an important role in regulating the absorption, transport, and storage of iron. • Mutations in these genes impair the control of iron absorption during digestion and alter the distribution of iron to other parts of the body. As a result, iron accumulates in tissues and organs. Incidence • Most common genetic disease in people of European ancestry • One in 8 are carriers of the single HH gene mutation. • Affects 1 in 200 to 500 in the United States • Very low prevalence in other ethnic populations Genetic Testing • DNA testing is available and recommended for all first-degree relatives of people with the disease. • American Hemochromatosis Society recommends genetic testing regardless of family history. • Useful diagnostic tests include serum iron concentration, total iron-binding capacity, and transferrin saturation. Clinical Implications • Most of the 33 million Americans who have the gene mutation do not know it. • Clinical expression is variable depending on dietary iron, blood loss, and other modifying factors. • Early treatment can prevent serious complications. • If untreated, progressive iron deposits can lead to multiple organ failure.
sickle cell disease genetics
Genetic Basis • Autosomal recessive disorder. • Mutation in β-globin (HBB) gene located on chromosome 11. • Various versions of β-globin result from different mutations in the HBB gene. • Hgb S variant involves substitution of valine for glutamic acid in the β-globin gene in sickle cell anemia. Incidence • Most common inherited blood disorder in the United States. • Affects 8 of every 100,000 people. • Affects 1 in 350 to 500 African Americans. • Affects 1 in 1000 to 1400 Hispanic Americans. • Also affects people of Mediterranean, Caribbean, Arabian, and East Indian descent. Genetic Testing • DNA testing is available. • Electrophoresis of hemoglobin and sickling screening test are more commonly used. Clinical Implications • Requires ongoing continuity of care and extensive patient teaching. • Sickle cell trait is the carrier state for sickle cell disease and represents a mild type of sickle cell disease; 1 in 10 to 12 African Americans and 1 in 25 Hispanics have sickle cell trait. • If both parents have the trait, there is a 1 in 4 chance that their baby will have sickle cell disease. • Management of sickle cell disease should focus on the prevention of sickle cell crisis (e.g., prevention of dehydration and infection). • Genetic counseling is recommended for individuals with a family history of sickle cell disease. Individuals should understand the risks of transmitting the genetic mutation.
genetics of hemophilia A and B
Genetic Basis • X-linked recessive disorder • Hemophilia A: Caused by mutations in the F8 gene that provides instructions for making coagulation factor VIII • Hemophilia B: Caused by mutations in the F9 gene that provides instructions for making coagulation factor IX • Mutations in the F8 or F9 gene lead to the production of an abnormal version or reduced amounts of these coagulation factors. Incidence • Hemophilia A: 1 in 5000 to 10,000 male births • Hemophilia B: 1 in 30,000 to 50,000 male births Genetic Testing • DNA testing is available. Clinical Implications • Female carriers transmit the genetic defect to 50% of their sons, and 50% of their daughters are carriers. • Males with hemophilia do not transmit the genetic defect to their sons, but all of their daughters are carriers. • Female hemophilia can occur if a male with hemophilia mates with a female carrier. However, this is a rare situation. • Clinical manifestations of hemophilia A and B are similar. • Replacement therapy is available for factors VIII and IX (Table 30-19).
Megakaryocytes
Giant cells in bone marrow that release platelets; enormous cells in the bone marrow that release packets of cytoplasm (platelets) into the circulating blood.
patient & caregiver teaching on neutropenia
Include the following instructions when teaching a patient or caregiver the precautions to take when the neutrophil count is low. 1. WASH YOUR HANDS frequently and make sure those around you wash their hands frequently, particularly if they help with your care. An antibacterial hand gel may also be used. 2. Notify your nurse or HCP if you have any of the following: • Fever ≥100.4° F (38° C)* • Chills or feeling hot • Redness, swelling, discharge, or new pain on or in your body • Changes in urination or bowel movements • Cough, sore throat, mouth sores, or blisters 3. If you are at home, take your temperature as directed and follow instructions on what to do if you have a fever. 4. Avoid crowds and people with colds, flu, or infections. If you are in a public area, wear a mask and use hand sanitizing gel frequently. 5. Avoid uncooked meats, seafood, or eggs and unwashed fruits and vegetables. Ask your HCP about specific dietary guidelines for you. 6. Bathe or shower daily. A moisturizer may be used to prevent skin from drying and cracking. 7. Maintain some daily activity as instructed by your health care team. This may include walking and moderate exercise while avoiding crowds. 8. Brush your teeth with a soft toothbrush four times daily. You may floss once daily if it does not cause excessive pain or bleeding. Avoid alcohol-based mouthwashes. 9. Do not perform gardening or clean up after pets. Feeding and petting your dog or cat are fine as long as you wash your hands well after handling.
patient & caregiver teaching on thrombocytopenia
Include the following instructions when teaching a patient or caregiver the precautions to take when the platelet count is low. 1. Notify your HCP of any manifestations of bleeding. These include the following: • Black, tarry, or bloody bowel movements • Black or bloody vomit, sputum, or urine • Bruising or small red or purple spots on the skin • Bleeding from the mouth or anywhere in the body • Headache or changes in how well you can see • Difficulty talking, sudden weakness of an arm or leg, confusion 2. Ask your HCP regarding restrictions in your normal activities, such as vigorous exercise or lifting weights. Generally, walking can be done safely and should be done with sturdy shoes or slippers. If you are weak and at risk for falling, get help or supervision when getting out of bed or chair. 3. Do not blow your nose forcefully; gently pat it with a tissue if needed. For a nosebleed, keep your head up and apply firm pressure to the nostrils and bridge of your nose. If bleeding continues, place an ice bag over the bridge of your nose and the nape of your neck. If you are unable to stop a nosebleed after 10 min, call your HCP. 4. Do not bend down with your head lower than your waist. 5. Prevent constipation by drinking plenty of fluids. Do not strain when having a bowel movement. Your HCP may prescribe a stool softener. Do not use a suppository, an enema, or a rectal thermometer without the permission of your HCP. 6. Shave only with an electric razor. Do not use blades. 7. Do not tweeze your eyebrows or other body hair. 8. Do not puncture your skin, such as getting tattoos or body piercing. 9. Avoid using any medication that can prolong bleeding, such as aspirin. Other medications and herbs can have similar effects. If you are unsure about any medication, ask your HCP or pharmacist about it in relation to your thrombocytopenia. 10. Use a soft-bristle toothbrush to prevent injuring the gums. Flossing is usually safe if it is done gently using the thin tape floss. Do not use alcohol-based mouthwashes, since they can dry your gums and increase bleeding. 11. Women who are menstruating should keep track of the number of pads that are used per day. When you start using more pads per day than usual or bleed more days, notify your HCP. Do not use tampons; only use sanitary pads. 12. Ask your HCP before you have any invasive procedures done, such as a dental cleaning, manicure, or pedicure.
causes of splenomegaly
Infections and Inflammations • Bacterial infections: endocarditis, salmonella • Mycobacterial infections: tuberculosis • Spirochetes: syphilis, Lyme disease • Viral infections: hepatitis, human immunodeficiency virus, cytomegalovirus, mononucleosis • Parasitic infections: malaria, trypanosomiasis, schistosomiasis, leishmaniasis, toxoplasmosis • Rickettsial infections: Rocky Mountain spotted fever, typhoid fever • Fungal infections: histoplasmosis • Autoimmune diseases: systemic lupus erythematosus, rheumatoid arthritis Infiltrative Diseases and Tumors or Cysts • Acute and chronic leukemia • Lymphomas • Polycythemia vera • Multiple myeloma, amyloidosis • Other primary or secondary neoplasms and cysts • Sarcoidosis • Gaucher disease Congestion • Cirrhosis of the liver • Heart failure (portal hypertension) • Portal or splenic vein thrombosis • Sickle cell disease • Thalassemia • Acquired hemolytic anemia • Immune thrombocytopenia
difference between leukopenia, granulocytopenia, & neutropenia
Leukopenia refers to a decrease in the total WBC count (granulocytes, monocytes, and lymphocytes). Granulocytopenia is a deficiency of granulocytes, which include neutrophils, eosinophils, and basophils. The neutrophilic granulocytes, which play a major role in phagocytizing pathogenic microbes, are closely monitored in clinical practice as an indicator of a patient's risk for infection. A reduction in neutrophils is termed neutropenia.
erythromelalgia
Painful burning and redness of the hands and feet
What is the most common infection that can occur in sickle cell disease patients?
Pneumonia
blood transfusions and roles
Registered Nurse (RN) • Ensure that an IV line is being used with a large-bore needle, catheter, or cannula, preferably 19 gauge or larger. • Double-check patient identification and blood product identification data with another licensed nurse (consider state nurse practice act and agency policy). • Adjust infusion rate of transfusion according to patient needs. • Evaluate patient for signs of transfusion reactions. • Evaluate for therapeutic effect of blood product (improvement in complete blood count, increased blood pressure, improved patient color, decreased bleeding). • Monitor for signs of circulatory overload (e.g., shortness of breath) if the transfusion must be given rapidly. Licensed Practical/Vocational Nurse (LPN/LVN) • Assist with checking patient identification and blood product identification data with the RN (consider state nurse practice act and agency policy). • Monitor blood transfusion rate (consider state nurse practice act and agency policy). Unlicensed Assistive Personnel (UAP) • Obtain blood products from the blood bank as directed by the RN. • Take vital signs before the transfusion and after the first 15 min. Role of Other Team Members Blood Bank Personnel • Responsible for typing and crossmatching the donor's blood with the recipient's blood. • Result of the compatibility testing should be noted on the product bag or tag. • When handing off the blood or blood component to nursing personnel, ensure that a positive identification is made with product and patient's information.
What is the primary site for RBC destruction?
The spleen. The spleen is the main site for RBCs destruction because they are old, defective, or moderately damaged.
True or false: folic acid is needed for DNA synthesis leading to RBC formation & maturation
True.
True or false: normal life span of an RBC is 120 days
True.
True or false: duodenum is involved in iron absorption.
True. So certain types of GI surgeries and malabsorption syndromes may cause malabsorption of iron, causing anemia.
True or false: RBC lysis can clog the kidney tubules and cause shock.
True. This is why aggressive hydration & electrolyte replacement is generally done to reduce risk of kidney injury caused by RBC lysis.
Cobalamin (B12)
Vital for blood formation and healthy nervous system
neutropenia
a decreased number of neutrophils -defined as ANC less than 1000 cell/uL -severe: ANC less than 500 cells/uL -most common cause: chemotherapy & immunosuppressive therapy
anemia
a deficiency of red blood cells (erythrocytes), the quantity or quality of hemoglobin, and/or volume of packed RBCs (hematocrit) -not a specific disease, just a manifestation of a pathologic process -diagnosed based on complete blood count (CBC), reticulocyte count, & peripheral blood smear -classified according to morphology or etiology (cause) -mild states: 60-100g/L
megaloblastic anemias
a group of disorders caused by impaired DNA synthesis and characterized by the presence of large red blood cells -since DNA synthesis is impaired, defective RBC maturation results -megaloblasts: large and abnormal RBCs -they are easily destroyed & have fragile membranes -mainly from cobalamin & folic acid deficiencies
sickle cell disease (SCD)
a group of inherited, autosomal recessive disorders characterized by the presence of an abnormal form of hemoglobin in the erythrocyte (RBC) -usually identified during neonatal screening -manifestations: generally healthy most of time except may have chronic health problems, asymptomatic except during sickling episodes, primary symptom is pain-accompanied by fever, swelling, tenderness, tachypnea, hypertension, nausea, & vomiting -complications: come from organs that have needs for large amounts of O2, infections due to failure of spleen to phagocytize foreign substances -diagnosis: peripheral blood smear, sickling test, findings of hemolysis (jaundice, elevated bilirubin), skeletal xrays -nursing management: > teach pts to avoid high altitudes, maintain adequate fluids, and treat infections > teach about screening for retinopathy > transfusion therapy needed when aplastic crisis occurs > pts w/ acute chest syndrome are treated w/ broad spectrum antibiotics, O2 therapy, fluid therapy, and possible exchange transfusion > hydroxyurea (Hydrea) only antisickling agent shown to be effective
myelodysplastic syndrome (MDS)
a group of related hematologic disorders characterized by a change in the quantity and quality of bone marrow elements; also characterized by peripheral blood cytopenia & changes in cellularity of the bone marrow with dysplastic changes -hematopoiesis is disorderly and ineffective -referred to as clonal disorder (because some bone marrow stem cells continue to function normally while some other do not) -one type may transform into another
sequelae
a problem resulting from a disease or injury
petechiae
a small red or purple spot caused by bleeding into the skin.
Disseminated Intravascular Coagulation (DIC)
abnormal activation of the proteins involved in blood coagulation, causing small blood clots to form in vessels and cutting off the supply of oxygen to distal tissues, abnormally initiated and accelerated clotting ,characterized by profuse bleeding that results from depletion of platelets and clotting factors -always caused by underlying disease or condition -etio/patho: not a disease but abnormal response to normal clotting cascade stimulated by disease process or disorder, can be acute, subacute, or chronic , tissue factor is released at site of tissue injury and some malignancies enhance normal coagulation mechanisms, leads to thrombosis & clotting inhibitory mechanisms are depressed *the excessive clotting activates the fibrinolytic system--> which in turn breakdown the newly formed clot--> FSPs accumulate & clotting factors are depleted--> blood loses ability to clot & stable clot cannot be formed at injury sites -manifestations: has both bleeding and thrombotic manifestations *bleeding: pallor, petechiae, purpura, oozing blood, venipuncture site bleeding, hematomas, occult hemorrhage, tachypnea, orthopnea, hypotension, bloody stools, hematuria, changes in mental status *thrombotic: cyanosis, ischmeic tissue necrosis, tachypnea, pulmonary emboli, ARDS, abdominal pain -diagnosis: as more clots are made in the body, more breakdown products from fibrinogen & fibrin form (called fibrin split products) -nursing management:
blood transfusion reaction
adverse reaction to blood transfusion that can range in severity from mild symptoms to life threatening -classified as acute or delayed -most common cause of hemolytic reactions is tranfusion of ABO incompatible blood -delayed transfusion reactions include delayed hemolytic reactions, infections, and iron overload
metrorrhagia
bleeding between periods
Von Willebrand Disease
bleeding disorder caused by a deficiency of von Willebrand factor, a "sticky" protein that lines blood vessels and reacts with platelets to form a plug that leads to clot formation; a related disorder to hemophilia -most common congenital bleeding disorder
hemarthrosis
blood within a joint
hemoptysis
bloody sputum
hemolytic anemia
condition caused by the destruction or hemolysis of RBCs at a rate that exceeds production -can occur because of intrinsic or extrinsic problems to the RBCs
multiple myeloma (plasma cell myeloma)
condition in which neoplastic cells infiltrate the bone marrow and destroy bone -more common in men and usually develops after 40 yrs -more common in African Americans -etio/patho: cause is unknown, possibly radiation, organic chemicals, metals, herbicides, insecticides, genetic factors, obesity *excessive production of plasma cells (activated B cells that produce antibodies to fight off infections) but monocolonal antibodies are produced (all one kind) so they become ineffective and harmful=infiltrate bone marrow *plasma cell production is excessive and abnormal amounts of cytokines are produced also=role in bone destruction -manifestations: develops slowly and insidiously, sx usually do not occur until symptoms is advanced, skeletal pain in most common, spinal cord may be compressed due to vertebral destruction, bone loss integrity *hypercalcemia (due to bone degeneration)= which may in turn cause seizures, coma, cardiac problems, anorexia, confusion *anemia, thrombocytopenia, neutropenia, immune dysfunction may occur -diagnosis: lab, radiologic, and bone marrow exam, MRI, PET, CT * M protein is usually found in blood & urine -nursing management: includes corticosteroids, chemotherapy, immunotherapy, targeted therapy, HSCT > ambulation & hydration are used to treat hypercalcemia, dehydration, and potential renal damage > kyphoplasty may be done to control spine vertebral disease > use caution when moving the patient since they are at risk of fractures > peripheral neuropathy may occur
hypersplenism
condition in which the spleen removes blood components at an excessive rate; occurence of splenomegaly and peripheral cytopenias
aplastic anemia
disease in which the pt has peripheral blood pancytopenia (decrease of all blood cell types) and hypocellular bone marrow -etiology: usually acquired, idiopathic & thought to have autoimmune basis, chemical agents/toxins, drugs, radiation, viral/bacterial infections -manifestations: can manifest abruptly over days and over weeks-to-months *general: fatigue, dyspnea, fever (considered medical emergency) -diagnostic studies: WBC, platelets, & hemoglobin generally decreased, reticulocyte count is low, condition is norocytic, normochromic anemia -nursing management: identifying and removing causative agent
splenomegaly
enlargement of the spleen
hemochromatosis
excess iron deposits throughout the body due to increased intestinal iron absorption so iron accumulates in tissues and organs -occurs primarily as a genetic defect but also can occur secondary to diseases -early s/sx: fatigue, arthralagia, impotence, abdominal pain, weight loss -later s/sx: liver enlargement--> cirrhosis (due to iron accumulation), skin pigment changes, cardiac changes, arthritis, testicular atrophy -labs: elevated iron, TIBC, ferritins, genetic mutation confirmation, liver biopsy -tx: removing iron by removing 500mL of blood each week for 2-3 yrs until storage is depleted--> then removed less frequently, iron chelating agents may be used, avoid vitamin C & iron
menorrhagia
excessive menstrual bleeding
nursing assessment of anemia
fatigue pallor low h&h poor nutritional intake noncompliance with prenatal vitamin and iron supplementation
leukemia
general term used to describe a group of malignant disorders affecting the blood and blood forming tissues of the bone marrow, lymph system, and spleen -results in an accumulation of dysfunctional cells because of loss of regulation in cell division -acute: characterized by the clonal proliferation of immature hematopoietic cells, develop after malignant transformation of a single type of immature hematopoietic cell
thalassemia
group of diseases involving inadequate production of normal hemoglobin and therefore decreased erythrocyte production -due to absent or reduced globulin protein -hemolysis also occurs
non-hodgkin's lymphomas (NHLs)
hetergeneous group of malignant neoplasms of primarily B- or T- or natural killer (TK) cell origin -B-cell lymphomas make up about 88% of all NHLs -classified based on morphologic, gneetic, immunophenotypic, clinical features -etio/patho: usually unknow, either chromosomal translocations, infections enrionmental facors, and immunodeficiency states, does not have hallmark feature (like Hodgkin's and Reed Ternberg cell) -manifestations: can originate outside of lymph nodes, spread method is unpredictable, primary sx is painless lymph node enlargement, sx depend on where disease has spread, may have fever, night sweats, weight loss -diagnosis: resemble those for Hodgkin's lymphoma, since more often in extranodal sites-more diagnostic studies may be done, MRI, lumbar puncture, bone marrow biopsy, upper endoscopy -nursing management: > more aggressive lymphomas are most responsive to treatment and more likely to be cured > manage problems related to the disease > know what specific organs are involved & how extensive it is
cheilitis
inflammation of the lips -third most common symptom of iron deficiency anemia
glossitis
inflammation of the tongue -second most common symptom of iron deficiency anemia
autotransfusion (autologous transfusion)
involves removing whole blood from person and transfusing it back in, includes... autologous donation (elective phlebotomy) (predeposit transfusion): donates before planned procedure, can be frozen & stored for up to 10 yrs autotransfusion: basically present time donating, usually during surgery *sometimes blood that has been collected has become depleted of its normal coagulation factors so monitor coagulation studies in the pts receiving autotransfusion
pernicious anemia
lack of mature erythrocytes caused by inability to absorb vitamin B12 into the bloodstream; type of megaloblastic anemia -gastric mucosa is not secreting IF & there is decrease in Hcl in the stomach -onset usually middle age or later, 60 yrs being most common diagnosis -most common in AA and Northern European ancestry -causes: GI surgery, Chrohn's disease, ileitis, celiac disease, excessive alcohol/hot tea ingestion, smoking, proton pump inhibitor (long term), strict vegetarians -manifestations: sore-red-beefy-shiny tongue, anorexia, nausea, vomiting, abdominal pain (due to tissue hypoxia), weakness, paresthesias of feet & hands, ataxia, impaired thought processes, reduced senses (manifestations may take several months) -diagnostic studies: reduced cobalamin levels
ecchymoses
larger blue or purplish patches on the skin (bruises)
thrombocytopenia
low platelet count; platelets below 150,000/uL -manifestations: many are asymptomatic, most major complication is hemorrhage *most common sx: bleeding, manifested as petechiae, purpura, superficial ecchymoses *internal bleeding s/sx: weakness, fainting, dizziness, tachycardia, abdominal pain, hypotension -diagnosis: platelet count is decreased, H & P, -nursing management: > platelet transfusions usually not recommended until counts are below 10,000/uL unless pt is actively bleeding > avoid aspirin & other platelet function drugs > corticosteroids may be used for ITP since it suppresses phagocytic response of macrophages > splenectomy may be indicated > for TTP, treat underlying disorder > avoid IM injections and subcut injections in these pts if possible
Hodgkin's lymphoma (Hodgkin's disease)
malignant condition characterized by proliferation of abnormal, giant, multinucleated cells (called Reed-Sternberg cells) which are located in lymph nodes -occurs most frequently in persons from 15-30 yrs and above 55 yrs -more common in men -etio/patho: cause still remains unknown, factors thought to cause are EBV, genetics, and exposure to occupational toxins, higher chance in HIV pts *lymph nodes are destroyed by monocytes/macrophages *eventually infiltrates other organs (especially lungs, spleen, & liver) -manifestations: usually insidious *first s/sx: enlargement of cervical, axillary, or inguinal lymph nodes, mediastinal node mass is second most common *other: weight loss, fatigue, weakness, fever, chills, tachycardia, night sweats, alcohol ingestion may complain of pain *later/advanced: hepatomegaly, splenomegaly, anemia, jaundice, spinal cord compression, bone pain -diagnosis: peripheral blood analysis, excisional lymph node biopsy, bone marrow exam, radiologic studies, PET w/CT used to stage disease -nursing management: > staging involves A or B classification, roman numeral (I to IV), and symptoms when disease is found > standard for chemotherapy is ABVD regimen > secondary cancers may occur 10 yrs after
lymphomas
malignant neoplasms originating into the bone marrow and lymphatic structures resulting in the proliferation of lymphocytes -2 major types 1) Hodgkin's lymphoma 2) non-Hodgkin's lymphoma (NHL)
purpura
multiple pinpoint hemorrhages and accumulation of blood under the skin
epistaxis
nosebleed
stages of chemotherapy
often divided into 3 stages 1) induction: an attempt to bring about remission -includes aggressive treatment to try & destroy leukemic cells and restore normal hematopoiesis -pt may become ill because bone marrow is severely depressed by chemo -nursing interventions: focus on neutropenia, thrombocytopenia, anemia -generally assumed that leukemic cells persist undetected after induction therapy=which could lead to relapse if no further therapy is administered 2) postinduction (postremission) (consolidation): includes intensification (high dose therapy) & consolidation (started after remission achieved and consists of additional courses of same drugs given during induction-meant to eliminate leukemic cells that may not be detectable) 3) maintenance: treatment with lower doses of same drugs used in induction or other drugs -given every 3-4 weeks for prolonged period -goal: keep body free of leukemic cells
intrinsic hemolytic anemia
refers to a defect in the RBC causing the anemia (membrane defects, abnormal Hb, enzyme deficiency); RBC defect; usually hereditary
acute chest syndrome
term used to describe acute pulmonary complications that include pneumonia, tissue infarction, & fat embolism -characterized by chest pain, cough, dyspnea
Autosplenectomy
when the spleen becomes small because of repeated scarring -phenomenon associated with sickle cell diseases
safety alert for neutropenia
• A low-grade fever in neutropenic patients is of great significance because it may indicate infection and lead to septic shock and death unless treated promptly. • Neutropenic fever (≥100.4° F [38° C] and a neutrophil count <500/µL) is a medical emergency. • Blood cultures should be drawn STAT and antibiotics started within 1 hr.
blood transfusions alerts
• Do not use dextrose solutions or lactated Ringer's solution for administering blood because they will cause RBC hemolysis. • Do not give any additives (including medications) via the same tubing as the blood unless the tubing is first cleared with saline solution.
causes of acquired thrombocytopenia
• Immune thrombocytopenic purpura (ITP) • Thrombotic thrombocytopenic purpura (TTP) • Heparin-induced thrombocytopenia (HIT) • Disseminated intravascular coagulation (DIC) • Drugs • Chemotherapy drugs • Aspirin • Quinine • Infections • Viral infection (hepatitis C virus, HIV, cytomegalovirus) • Bacterial infection • Hematologic malignancy (leukemia, lymphoma, myeloma)
hematologic problems among cultures & ethnics
• Sickle cell disease has a high incidence among African Americans. • Thalassemia has a high incidence among African Americans and people of Mediterranean origin. • Tay-Sachs disease has the highest incidence in families of Eastern European Jewish origin, especially the Ashkenazi Jews. • Pernicious anemia has a high incidence among Scandinavians and African Americans.
