1 Hematology Review Questions 1. What is the unique nature of a stem cell and what does it mean to be committed? a.

The bone marrow first produces stem cells. These blood stem cells are immature, unspecialized (undifferentiated) cells that are capable of becoming any one of several types of blood cells: RBCs, WBCs, or platelets, depending on the bodys needs. b. The next stage in blood cell production is the committed stem cell (precursor cell). A committed stem cell enters one growth pathway and can at that point specialize (differentiate) into only one cell type. i. Committed stem cells actively divide but require a specific growth factor for specialization. 2. What is the difference between intrinsic and extrinsic clotting factors? a. Intrinsic clotting factorsproblems or substances directly in the blood itself that first make platelets clump and then trigger the blood clotting cascade. i. Circulating debris, prolonged venous stasis b. Extrinsic clotting factorsoutside of the cell than can induce platelet plugs to form. i. Trauma, inflammation, bacterial toxins, or foreign proteins ii. The blood-clotting cascade is started sooner by this pathway because some of the steps of the intrinsic pathway are bypassed.

3. What is the difference between anticoagulants and thrombolytics? a. Anticoagulants- work by interfering with one or more steps involved in the bloodclotting cascade. So they prevent new clots from forming and limit or prevent extension of formed clots. Anticoagulants cannot break down existing clots. b. Thrombolytics- break up existing clots. i. First line therapy for problems caused by small, localized, formed clots such as myocardial infarction (MI), limited arterial thrombosis, and thrombotic strokes. 4. What lab values are evaluated to determine coagulation status? What are their normal ranges? a. CBC w/ or w/o differential i. RBCs: males 4.7-6.1 million/mm3 females 4.2-5.4 million/mm3 ii. WBCs: 5,000-10,000/mm3 iii. Platelets: 150,000-400,000/mm3 iv. Hemoglobin: male (14-18 g/dL), female (12-16 g/dL) v. Hematocrit: males (42-52%), females (37-47%) vi. Reticulocyte Count: <2% of circulating RBCs (immature RBCs) vii. Prothrombin Time (PT: 11-12.5 seconds (time it takes for blood to clot) viii. International Normalized Ratio (INR): goal of 2-3 secs for patients on coumadin therapy ix. Partial Thromboplastin Time (PTT): 20-40 seconds depending on lab x. Coombs Test: should be negative 1. Positive findings indicate antibodies to RBCs

2 5. Discuss the procedure behind bone marrow biopsies- describe important post-procedure assessment and nursing care a. Performed to evaluate the patients hematologic status when other tests show persistent abnormal findings. b. Invasive procedure requiring informed consent c. Bone marrow aspiration- cells and fluids suctioned from marrow d. Bone marrow biopsy- solid tissue and cells obtained by coring out an area of bone marrow with a large bore needle. e. Nursing interventions: i. Local anesthetic as well as sedative (Versed, Ativan) ii. Monitor closely for 24 hours for bleeding/infection, Cover site with dressing, mild analgesic, Ice packs, avoid contact to area iii. Dont give aspirin or Motrin 6. What are the classic assessment findings in the client with anemia? Pallor, cool skin, brittle nails, Decreased oxygen saturation, SOB, dyspnea, tachycardia, orthostatic hypotension, fatigue, headaches 7. How does one develop SSA and what are some triggers contributing to a flare-up? Discuss methods of prevention and treatment of flare-ups SSA is an Autosomal recessive genetic disorder- results in chronic anemia, pain, disability, organ damage, increase risk for infection, early death. Triggers: Hypoxia, Dehydration, Infection, Venous stasis, Pregnancy, Alcohol use, High altitudes, Low environment or body temperature, Acidosis, Strenuous exercise, Anesthesia 8. How does iron deficiency anemia develop and who is at an increased risk for its development? Development: In 3 stages: Iron stores in body decreased, Iron stores in hemoglobin depleted, Insufficient iron for developing RBCs (Hgb deficient RBCs). Anyone who is malnourished, has a low dietary intake of iron or an increased need for iron or excessive blood loss. 9. What is the significance of folic acid in RBC production? a. Production of RBCs depends on adequate DNA synthesis in the precursor cell so that cell division and growth into functional RBCs can occur. All cell division requires adequate amounts of folic acid to make DNA. Vitamin B12 moves folic acid into cells so DNA synthesis can occur.

10. How is folic acid deficiency and vitamin B12 deficiency anemia differentiated? a. Nervous system functions remain normal because folic acid deficiency does not affect nerve function. Absence of neurological problems in folic acid deficiency anemia marks the only difference between these two anemias.

11. What is pancytopenia and in what disease states is it common? a. Pancytopenia is a deficiency of all three cell types causing aplastic anemia, leukopenia, and thrombocytopenia. i. Aplastic anemia is a deficiency of circulating RBCs because of failure of the bone marrow to produce these cells.

3 1. Caused by an injury to the pluripotent stem cell (immature precursor cell for RBCs), which can be caused by long-term exposure to toxic agents and drugs, ionizing radiation, or infection. Many times reason is unknown.

12. Why is someone with polycythemia vera at risk for tissue hypoxia? a. PV is a cancer of the RBCs with an increase in number of circulating RBCs leading to hyperviscous blood. 3 hallmarks: i. Massive production of RBCs ii. Excessive leukocyte production iii. Excessive production of platelets b. In some highly vascular areas, blood flow may become so slow that stasis occurs. Vascular stasis causes thrombosis (clot formation) within the smaller vessels, occluding blood vessels. The blood vessel occlusion leads to tissue hypoxia, anoxia, and later to infarction and necrosis. c. Shorter life spans and increased cell production cause a rapid turnover of circulating blood cells which leads to increasing amounts of cell debris (released when cells die) in the blood, adding to the general sludging of the blood. This debris includes uric acid and potassium, which causes symptoms of gout and hyperkalemia. d. Manifestations of PV: facial skin and mucous membranes dark and flushed with purple/cyanotic appearance, intense itching, HTN, risk for thrombosis, risk for gout and hyperkalemia, bleeding

13. What is the difference between lymphocytic and myelogenous leukemia? a. Leukemias are classified by cell type i. From the lymphoid pathway: lymphocytic or lymphoblastic ii. From the myeloid pathway: myelocytic or myelogenous b. The presence of the Philadelphia chromosome is the hallmark of chronic myelogenous leukemia (CML) c. Cancer with uncontrolled production of immature WBCs in bone marrow. Clinical manifestations of leukemia are most related to bone marrow depression and effects of decreased tissue perfusion r/t anemia. d. Primary ND: increased risk of infection and bleeding

14. What is the standard treatment for leukemia and what are the associated complications? Why may a client who has received previous treatment for cancer develop a different type of cancer later in life? a. Drug therapy for Acute Leukemia: i. Induction therapy intense and consists of combination chemotherapy (cytosine arbinoside and daunorubicin). Goal is to achieve a rapid, complete remission of all manifestations of disease. Prolonged hospitalizations are common while the patient is immunosuppressed. SE: N/V, diarrhea, alopecia (hair loss), stomatitis (mouth sores), kidney toxicity, liver toxicity, and cardiac toxicity.

4 ii. Consolidation therapytreatment occurs early in remission and its intent is to cure. iii. Maintenance therapyprescribed for months to years after successful induction and consolidation therapies. Purpose is to maintain the remission achieved. b. Standard treatment for patients with Leukemia is a bone marrow transplant (hematopoietic stem cell transplant). This is done by ridding client of leukemic cells via high dose chemotherapy and sometimes total body irradiation and then performing the transplant. i. Complications: 1. Bleeding and infection 2. Failure to engraftdonated stem cells fail to grow in the bone marrow and function properly. 3. Graft vs. host diseasemostly in allogenic (from sibling or matched unrelated donor) transplants. The immunocompetent cells of the donated marrow recognize the patients (recipients) cells, tissues, and organs as foreign and start an immunologic attack against them. The graft is actually trying to attack the host tissue and cells. 4. Veno-occlusive diseasethe blockage of liver blood vessels by clotting and inflammation. Symptoms of liver failure- right upper quadrant abdominal pain, jaundice, ascites, weight gain, and liver enlargement.

15. Discuss the process of bone marrow transplantation. What does it mean to engraft? discuss potential complications associated with BMTs and possible treatments for each

16. What are the main differences in diagnosing Hodgkins and non-Hodgkins Lymphoma? a. Hodgkins and non-Hodgkins lymphoma are characterized by abnormal proliferation of lymphocytes. b. Hodgkins lymphoma is a malignancy of the lymph nodes that originates in a single lymph node or chain of nodes. i. Spreads in an orderly fashion. ii. Distinguishing featureReed-Sternberg (RS) cells iii. May be caused by viral infections: EBV, HIV or chemical agents iv. Clinical manifestations: enlarged, painless mass in neck often first sign, mediastinal mass found on X-ray, lymphadenopathy, B symptoms 1. B symptomsintermittent fever, drenching night sweats, weight loss, anemia v. Diagnosis through node biopsy and presence of Reed-Sternberg (RS) cells. vi. Treatment includes radiation in earlier stages and combination chemotherapy for later stages. c. Non-Hodgkins lymphoma includes all lymphoid cancers that do not have the ReedSternberg cell. i. Clinical manifestations: lymphadenopathy and extranodal involvement (lesions of bone, GI tract, skin, and CNS) 1. Does not spread through lymphatic system in orderly fashion 2. Presence of B symptoms less common

5 ii. Treatment includes radiation, chemotherapy, and even bone marrow transplant because there is nodal involvement. 17. Describe the pathophysiology behind multiple myeloma. How is multiple myeloma typically diagnosed and treated? a. Cancer of mature WBC. There is an overgrowth of B-lymphocyte plasma cells in the bone marrow. These cells normally make antibodies. When they become cancerous these plasma cells overproduce antibodies (gamma globulins). Thus the disorder is called a gammopathy. i. The abnormal plasma cells produce an abnormal antibody (myeloma protein or the Bence Jones protein) found in the blood and urine. ii. Multiple myeloma cells also produce excess cytokines that increase cancer cell growth rates and destroy bone. iii. Causes decreased production of immunoglobulin and antibodies and increased levels of uric acid and calcium, which can lead to renal failure. b. Assessment: i. Bone (skeletal) pain especially in the ribs, spine, and pelvis ii. Weakness and fatigue iii. Recurrent infections iv. Anemia v. Osteoporosis & fractures vi. Thrombocytopenia and leukopenia vii. Elevated calcium and uric acid levels viii. Renal failure ix. Spinal cord compression and paraplegia c. Diagnosis i. Bone X-raySwiss cheese ii. Urinalysis shows Bence Jones proteinuria and elevated total serum protein level iii. Elevated immunoglobulin levels iv. Biopsy of lesions d. Treatment i. Chemotherapy (dexamethasone w/ or w/out thalidomide or bortezomib) 1. Thalidomide causes severe birth defects ii. Plasmapheresis (to selectively take out bad cells) and bone marrow transplant iii. Bisphosphanates to slow bone damage and reduce pain and risk of fractures

18. What are the main differences between autoimmune and thrombotic thrombocytopenia purpura? How are each treated? a. Autoimmune thrombocytopenianumber of circulating platelets reduced with normal bone marrow production. Patients make an antibody directed against the surface of their own platelets (an antiplatelet antibody). i. The antibody coats the surface of the platelets, making them more likely to be destroyed by macrophages. ii. Treatment: prevent bleeding episodes!!! 1. Platelet transfusion- used when platelet count <20,000/mm3 2. Steroids- azathioprine (Imuran) used to inhibit immune system production of antiplatelet autoantibodies 3. IV Immunoglobulin- prevent destruction of antibody coated platelets 4. Splenectomy- post-op patient at increased risk for infection

6 b. Thrombotic thrombocytopeniaplatelets clump together in capillaries with too few remaining in circulation. Clumping results in vessel occlusion and ischemia, leading to MI, stroke, and renal failure. i. Treatment: prevent platelet clumping and stop the underlying autoimmune process 1. Plasmapheresis and the infusion of fresh frozen plasma 2. Meds to inhibit platelet clumping- aspirin, alprostadil (Prostin) 19. What is the pathophysiology behind hemophilia? What are the main differences between Hemophilia A & B and how is each treated? a. Hemophiliahereditary bleeding disorder resulting from deficiencies in clotting factors i. Sex-linked disease (X-linked recessive trait) ii. Hemophilia A (classic)- deficiency of factor VIII (80%) iii. Hemophilia B (Christmas)- deficiency of factor IX (20%) b. Assessment: excessive bleeding from minor cuts, bruises, or abrasions (from abnormal platelet function), easily bruise, pain from chronic bleeding into joints, abnormal bleeding for longer period of time c. Labs: prolonged partial thromboplastin time (PTT) and a normal prothrombin time (PT/INR) d. Treatment: a regularly scheduled and/or intermittent infusions of factor VIII cryoprecipitate and blood transfusions

20. Discuss when various types of blood component therapy would be indicated. What are important nursing consideration pre, during, and post-transfusion? a. Indications of blood transfusions: active bleeding i. PRBCsusually for Hgb <8g/dL or hypoxemic; transplantation of tissue (require ABO and Rh antigen compatibility check ii. Platelets-- <10,000 thrombocytopenia with active bleeding, scheduled for invasive surgery 1. Pooled from donors: dont need same blood type as patient 2. Infuse over 15-30 minutes iii. Fresh Frozen Plasmareplace blood volume, active clotting disorders, PT or PTT >1.5 times normal 1. ABO compatibility required 2. Infuse over 30-60 minutes iv. Cryoprecipitate product derived from plasma; deficiency in clotting factor VIII (hemophilia A), presence of von Willebrands factor 1. Usually given IVP when fibrinogen level <100 mg/dL 2. Best if ABO compatible b. Pre-transfusion nursing considerations: i. Verify order/ get consent ii. Type and cross match iii. 20 gauge IV and tubing with blood filter iv. 0.9NS flush (others may cause clotting) v. Check blood product (pt ID, ABO, Rh, expiration date) vi. Vital signs taken immediately prior to transfusion- w/in 15 minutes of starting transfusion c. During transfusion: i. Vital signs after 15 minutes and then every hour

7 ii. Stay with patient for at least 15-30 minutes iii. Fluid overload is a potential complication of rapid infusion iv. Assess for chills, SOB, hives, itching, raise in temperature v. 1 unit= 2 hours minimum, 4 hours maximum (blood contaminated after 4 hours) d. After transfusion: i. Discontinue infusion and dispose of the bag and tubing properly ii. Document 21. Discuss the several types of transfusion reactions, why each may occur, and relevant nursing considerations a. Febrileoccurs most often in the patient with anti-WBC antibodies, a situation that can develop after multiple transfusions. i. S&S- chills, tachycardia, fever, hypotension, and tachypnea b. Hemolyticcaused by blood type or Rh incompatibility i. Antigen-antibody complexes that destroy transfused cells and start inflammatory response. ii. Reaction may be mild with fever and chills or life threatening with disseminated intravascular coagulation (DIC) and circulatory collapse. c. Bacterialoccur as a result of infusion of contaminated blood products. i. S&S- tachycardia, hypotension, fever, chills, and shock, onset is quick d. Allergicoccurs up to 24 hours post transfusion; most often in patients with history of allergies i. S&S: urticaria (hives), itching, bronshospasm, or anaphylaxis ii. Prevent this reaction by giving the patient washed RBCs e. Circulatory overloadoccurs when a blood product is infused too quickly. Most common with whole blood transfusions or when the patient receives multiple transfusions. Older adults most at risk for this. i. S&S: HTN, bounding pulse, distended jugular veins, dyspnea, restlessness, confusion ii. Manage and prevent this complication by monitoring intake and output, infusing blood products more slowly, and giving diuretics (doses of Lasix in between transfusions) f. Graft vs. Host diseaseimmunosuppressed patients, donor T-cell lymphocytes attack host tissues. i. Manifestations occur within 1-2 weeks and include thrombocytopenia, anorexia, N/V, chronic hepatitis, weight loss, and recurrent infection ii. 80-90% mortality rate but can be prevented by using irradiated blood products. Irradiation destroys T-cells and their cytokine products.