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Here's a few questions to help with studying for M2 tests and/or the USMLE. This will be continually updated with new questions, so check back soon! Find a mistake? Email dlynch@creighton.edu Links: http://teachingcases.hematology.org/
-Hematology Questions-
Questions 1-5 1. The object indicted by the arrow is most likely which of the following?
A. Plasmodium falciparum B. Heinz body C. hemoglobin C crystal D. polymerized hemoglobin S E. nuclear remnant
2. Which two of the following are necessary for the initiation of hemoglobin synthesis? A. histidine B. glycine C. succinyl CoA D. folate E. iron
3. On a peripheral blood smear you notice numerous round, dense RBCs that lack central pallor. Which of the following is a feature of hereditary spherocytosis? A. intravascular hemolysis of RBCs B. resistant to hypotonic solutions C. decreased mean corpuscular hemoglobin concentration (MCHC) D. splenectomy is often the best treatment E. mutation in spectrin
4. The following blood smear was taken from a pregnant 30 year woman, gravida 5 para 4. She is is her second trimester and complaining of fatigue. What is the most likely diagnosis?
A. B12 deficiency B. folate deficiency C. iron deficiency D. sickle cell anemia E. CML
5. The deficiency of the glycosylphosphatidylinositol (GPI) anchor in Paroxysmal Nocturnal Hemoglobinuria is significant because: A. GPI normally links to proteins which inactivate complement, and a deficiency of GPI leads to complement-mediated lysis B. mutant GPI is antigenic, leading to autoantibodies C. a deficiency of GPI causes RBCs to become rigid, and rigid RBCs lead to infarction D. GPI is unrelated to the pathogenesis of PNH
Answers 1-5 1. Answer E The object is a Howell-Jolly body, which is a remnant of the nucleus. After the spleen becomes dysfunctional in a person with sickle cell anemia, the nucleus is not completely removed from new RBCs. Howell-Jolly bodies are a sign of splenic dysfuntion, so they are not exclusive to sickle cell anemia. A Heinz body is more likely to be seen in a person with Glucose-6-phosphate dehydrogenase deficiency. These will appear as multiple dark bodies within a RBC, and need to be stained with crystal violet.
2. Answers B&C
3. Answer D Hereditary sphereocytosis is an autosomal dominant disorder that results in extravascular hemolysis of RBCs. The central defect is usualy a mutation in ankrin, which is a protein partially responsible for maintaining the biconcave shape of normal RBCs. If the mutation is in spectrin, the patient will likely have hereditary elliptocytosis. Treatment in both cases is usually a splectomy to prevent extravascular hemolysis in the spleen of the defective RBCs.
4. Answer C Notice the microcytic nature of the RBCs have increases central pallor. This rules out a B12 or folate deficiency. Sickle cell is not apparent, and the neutrophil looks normal. Women run the risk of becoming iron deficient with multiple pregnancies, particularly if they are breastfeeding.
5. Answer A The fundamental mutation in PNH is in PIGA, which is necessary for synthesis of GPI. GPI normally is linked to decay-accelerating factor (DAF) and membrane inhibitor of reactive lysis (CD59) which act to inhibit complement.
Questions 6-10 6. An 18 month old infant presents with persistent lesions on the scalp, hepatosplenomegaly, and swollen lymph nodes. Which of the following is the most appropriate test to diagnose the infant with Langerhans cell histiocytosis? A. CBC B. peripheral blood smear C. electron microscopy D. MRI E. CT F. culture of the lesions
7. Destruction of the bone marrow will likely result in which of the following features on a peripheral blood smear? A. teardrop-shaped red cells and large nucleated erythroid precursors B. increased WBC count and bite cells C. Heinz bodies in RBCs and smudge cells D. decreased reticulocyte count and hepatosplenomegaly
8. Thromboxane A2 is primarily: A. formed in platelets and acts as a vasoconstrictor B. formed in platelets and acts as a vasodilator C. formed in RBCs and acts as a vasoconstrictor D. formed in RBCs and acts as a vasodilator E. formed in WBCs and acts as a vasoconstrictor
9. Which of the following opposes the actions of Thromboxane A2, and where is it produced? A. Prostacyclin (PGI2) formed by WBCs B. Leukotrienes formed by the endothelium C. Prostacyclin (PGI2) formed by the endothelium D. Leukotrienes formed in WBCs
10. Pluripotent stem cells give rise to a myeloid line and a lymphoid line of cells. Which of the following is not a product of the myeloid cell line? A. erythrocyte B. neutrophil C. platelet D. natural killer (NK) cell E. monocyte F. eosinophil
Answers 6-10 6. Answer C Langerhans histiocytosis can present in a variety of way. Worth remembering is that it is more common in children with skin lesions and organomegaly. The EM of a lesion will show Birbeck granules, which look like tennis raquets.
7. Answer A As the bone marrow becomes incapable of producing blood cells, the spleen and liver will begin hematopoiesis. RBC produced at these locations tend to be teardrop-shaped.
8. Answer A In addition to vasoconstriction, Thromboxane A2 also causes platelet aggregation. It is derived from prostaglandins in the cyclooxygenase (COX) pathway. So, where would you find the highest concentrations of thromboxane synthase? platelets.
9. Answer C Both PGI2 and TxA2 are products of the COX pathway. TxA2 is produced by platelets to cause vasoconstriction and platelet aggregation, and PGI2 is produced in the endothelium with opposing effects. Leukotrienes are products of neutrophils in the lipoxygenase pathway and promote vasoconstriction as well as vascular permeability.
10. Answer D The lymphoid cell line produces B cells, T cells, and NK cells. Its easiest to remember these three and know that everything else is myeloid. The B and NK cells mature in the bone marrow while T cells mature in the thymus.
Questions 11-15 11. Hemoglobin F is increased in β-Thalassemia major. Which of the following shows the proper tetramer for HbF? A. ζ2ε2 B. α2β2 C. α2γ2 D. α2ε2 E. α2δ2
12. The fundamental change that causes Hemoglobin S is A. a mutation on the sixth position of the β chain of hemoglobin from Glu to Val B. a mutation on the sixth position of the β chain of hemoglobin from Leu to His C. a mutation on the sixth position of the α chain of hemoglobin from Glu to Val D. a mutation on the sixth position of the α chain of hemoglobin from Leu to His
13. Chronic myelogenous leukemia is largely caused by A. c-myc on chromosome 22 B. c-myc on chromosome 9 C. bcr/abl on chromosome 9 D. bcr/abl on chromosome 22
15. The smear shown below is an example of
A. microcytic anemia B. macrocytic anemia C. normocytic anemia D. thrombocytopenia E. normal blood
15. The following blood smear was most likely taken from a patient with
A. t(9:22) bcr abl B. t(8;14) c-myc C. t(14;18) bcl-2 D. Mantle cell lymphoma
Answers 11-15 11. Answer C A. ζ2ε2 = Gower-1, an embryonic Hgb (ε as in εmbryo) B. α2β2 = Hgb A, what most people have C. α2γ2 = Hgb F, why babies can survive in utero D. α2ε2 = Gower-2, another embyonic Hgb E. α2δ2 = Hgb A2, normally seen in small amounts in normal individuals, and may or may not be increased in β-Thalassemia major
12. Answer A This shift moves from the anionic glu to the hydrophobic valine. Electrophoresis can take advantage of this change in charge to diagnose Hgb S. The sickle cell hemoglobin will not migrate as far to the positive electrode as compared to normal Hgb.
13. Answer D The bcr/abl fusion product on chromosome 22 (from chromosome 9) is a tyrosine kinase whose activity results in CML.
14. Answer E. This is normal blood.
15. Answer A The smear shows neutrophils at all different stages of development (myeloblasts, myelocytes, metamyelocytes, band neutrophils), a key feature of CML. Basophils are often elevated as well. The c-myc translocation is found in Burkitt's lymphoma. It has a starry sky appearane on histology. It is associated with EBC and most often occurs in children. The bcl-2 activation occurs in follicular lymphomas. Mantle cell lymphomas are comprised of B cells and typically have a t(11;14) translocation. They are CD5 positive.
Questions 16-20 16. The site of action of warfarin is in A. blood clots B. platelets C. the liver D. the stomach E. bone marrow
17. Which is not a potential toxicity from the ABVD chemotherapy regimen used to treat Hodgkin's lymphoma? A. nephrotoxicity B. pulmonary fibrosis C. bone marrow toxicity D. cardiotoxicity
18. A patient has a CBC with erythrocytosis and thrombocytosis. Erythropoeietin levels are decreased, and plasma volume is on the high end of normal. Which of the following is most likely in this patient? A. JAK2 mutation B. renal disease C. COPD D. dehydration E. paraneoplastic syndrome
19. Which of the following is the best distinguishing factor between CML and a leukemoid reaction? A. WBC count of 50,000/μL B. lack of promyelocytes C. presence of eosinophils D. tear drop RBCs
20. A 4 year old child is having trouble in school. His teacher describes his behavior as 'distracted and erratic'. Lab values indicate a hypochromic, microcytic anemia, and a blood smear shows multiple basophilic specks on a small portion of red blood cells. Which of the following is the most likely complication of the poisoning the child is experiencing? A. accelerated growth B. infertility C. hyperdense bones on Xray D. hypotension
Answers 16-20 16. Answer C Warfarin is a vitamin K antagonist. It acts by inhibiting the synthesis of vitamin K dependent clotting factors, such as factors II, V, IX and X. These are synthesized in the liver.
17. Answer A ABVD is the combination of adriamycin, bleomycin, vinblastine, and dacarbazine. Here's a mnemonic to help remember the toxicities. Another important fact to remember is that Vincristine does not have bone marrow toxicity.
18. Answer A This case describes polycythemia vera, where there is a JAK2 mutation causing increased response to eythropoietin. Renal disease, COPD, and paraneoplastic syndrome could cause secondary polycythemia from excess erythropoietin production. These are ruled out since erythropoietin levels were normal. Dehydration causes a decrease in plasma volume, resulting in the appearance of elevated RBC volume. This is relative, or spurious polycythemia.
19. Answer B A leukemoid reaction is an exaggerated reponse to an infection. WBC counts are elevated and eosinophils are present in both CML and a leukemoid reaction. However, there will be no promyelocytes in a leukemoid reaction. CML is characterized by neutrophils at all stages of development, so it will have promyelocytes. Tear drop RBCs are a characteristic of RBC production outside the bone marrow. This could happen in CML or leukemoid reactions.
20. Answer C Lead is stored in the bones, resulting in hyperdense bones.
Questions 21-25 21. A mother brings her 15 month old son to you and complains that he bled too much after scraping his knee. You find that he has a prolonged bleeding time, prolonged PTT, and normal PT. He has a normal platelet count as well. What is the likely diagnosis? A. Hemophilia A B. Christmas disease C. DIC D. Bernard-Soulier disease E. vitamin K deficiency F. von Willebrand disease
22. A patient presents to the ER with head trauma. An x-ray of the skull reveals no fracture, but the skull appears to have a "crew cut" appearance. Which of the following conditions could result in this finding? A. microangiopathic hemolytic anemia B. hereditary spherocytosis C. α-thalassemia D. sickle cell anemia E. iron deficiency
23. A 50 year old man comes in for a routine physical. The doctor finds a systolic crecendo-decrecendo murmur heard loudest on the right sternal border. Labs reveal decreased hemoglobin and hematocrit. How are these two findings most likely to be related? A. autoimmunity B. microangiopathic hemolytic anemia C. Chagas disease D. normal aging
24. Which of the following conditions will result in an increased transferrin saturation? A. hemochromatosis B. anemia of chronic disease C. iron deficiency anemia D. α-thalassemia
25. What type of lymphoma comes from cells indicated by the arrow?
A. T cell B. B cell C. histiocytic
Answers 21-25 21. Answer F When bleeding time and PTT are elevated, think von Willebrand. He doesn't have Hemophilia because his bleeding time is normal. The only two that elevate PT are DIC and vitamin K deficiency, so those are eliminated
22. Answer D Increased bone marrow in the skull causes the 'crew cut' appearance on x-rays. This is most likely to occur in β-thalassemia and sickle cell anemia.
23. Answer B The murmur described here is aortic stenosis, which can be caused by a congenital bicuspid valve that becomes calcified. This dystrophic material on the valve destroys red blood cells, causing anemia. This is a tough question if you haven't had cardio yet, but it's worth knowing causes of microangiopathic hemolytic anemia.
24. Answer A Hemochromatosis is an autosomal recessive disorder of iron overload. Iron is deposited throughout the body, mostly the liver, heart, pancreas, joints, and pituitary gland. Since there is too much iron, it makes sense that transferrin (the molecule that carries iron) has increased saturation. Hemochromatosis makes for very good test questions, so make sure you spend some time learning it. It presents at "bronze diabetes", since the iron gives color to the skin and interferes with pancreatic function. You might see a biopsy of the liver stained with Prussian blue, which shows iron deposition.
25. Answer B B cell lymphomas come from the germinal follicles. T cell lymphomas come from the paracortix (the area around the follicle). Histiocytic lymphomas come from the sinuses of the lymph nodes (the periphery of the lymph node).
Questions 26-30 26. In evaluation of a patient with a bleeding disorder, the only abnormality found is an elevated bleeding time. Platelet count, PT and PTT are normal. What drug could cause this same effect? A. warfarin B. heparin C. abciximab D. lepirudin E. methotrexate
27. The medial cubital vein is often used for blood draws. What nerve and artery are most susceptible to injury during this process? A. median n., branchial a. B. median n., radial a. C. ulnar n., branchial a. D. ulnar n., interosseous a. E. musculocutaneous n, branchial a. F. musculocutaneous n, ulnar a.
28. Which of the following is a T cell Non-Hodgkin's lymphomas? A. Burkitt's lymphoma B. lymphoblastic lymphoma C. mantle cell lymphoma D. small lymphocytic lymphoma E. follicular lymphoma
29. Which of the following is directly responsible for the D-dimers seen in disseminated intravascular coagulation? A. tPA B. fibrinogen C. plasmin D. factor XIII
30. "Bridging" is a term used when heparin is used to begin anticoagulation and then coumadin is used for maintainance. Which of the following is the best explanation for why this is done? A. heparin breaks down clots that may have already formed B. heparin is more potent than coumadin C. coumadin takes weeks to begin working D. heparin is required to activate coumadin E. coumadin is actually thrombogenic when first administered
Answers 26-30 26. Answer C Since there is only an abnormality in bleeding time, there must be a problem with this patient's platelets. He likely has Glanzmann's thrombasthenia. There are a number of drugs that would cause this same effect: aspirin, clopidogrel, and abciximab. All decrease platelet aggregation without affecting the clotting cascade. Clopidogrel blocks ADP receptors to decrease GPIIb/IIIa expression, whereas abciximab directly binds GPIIb/IIIa. Aspirin inhibits both COX 1 and 2 pathways. Its antiplatelet activity comes from reducing the amount of thromboxane A2 produced. Methotrexate is an anticancer drug that inhibits dihydrofolate reductase, stopping cells in S phase due to decreased DNA and protein synthesis.
27. Answer A
28. Answer B Lymphoblastic lymphoma is an aggressive T cell lymphoma seen in children.
29. Answer C Plasmin cleaves fibrinogen to make fibrin split products (d-dimers). tPA converts plasminogen to plasmin.
30. Answer E Coumadin can cause clots when first administered, so patients are bridged with heparin until the coumadin is therapeutic, about 2-3 days.
Questions 31-35 31. Which of the following serum changes are most likely in a patient with Hodgkin’s lymphoma who was recently treated with CHOP therapy?
32.What prophylaxis could have minimized these changes in the patient in problem 31? A. IV fluids B. allopurinol C. vitamin B12 D. A and B E. A and C F. A, B and C
33. Auer rods are observed on the peripheral smear of a patient with constant bleeding. His PT and PTT are increased, his fibrinogen is decreased, and his fibrin split products are increased. What type of leukemia is he most likely to have? A. CML B. AML type M1 C. AML type M2 D. AML type M3 E. AML type M5 F. CLL G. ALL
34. Which is most indicative of disseminated intravascular coagulopathy, or DIC?
35. Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are similar conditions. Which of the following findings indicates TTP over HUS? A. positive E. coli O157:H7 culture B. increased creatinine C. confusion and headache D. schistocytes E. Howell-Jolly bodies
Answers 31-35 31. Answer A The administration of chemotherapy can cause tumor lysis syndrome. The intracellular fluid is high in potassium, uric acid, and phosphate. As cells die in response to chemotherapy, they release these into the plasma, raising the total levels. The decrease in calcium is a secondary effect of the hyperphosphatemia. The phosphate is a highly negative ion which binds the positively charged calcium already in the plasma.
32. Answer D The changes will be minimized by keeping the patient well hydrated. Uric acid synthesis can be reduced with allopurinol. This medication is also used to prevent gout attacks, which are caused by uric acid crystal formation in joint spaces.
33. Answer D Auer rods are diagnostic of acute myelogenous leukemia, or AML. Therefore, the question is asking which subtype of AML presents in this way. AML type M3 is known to present with DIC, or disseminated intravascular coagulation. DIC is characterized by the consumption of all clotting factors (manifested by increased PT and PTT and decreased fibrinogen) and reduced platelets. As clots form and are broken down, fibrin split products are produced.
34. Answer E DIC uses up everything involved in clotting, including platelets. As RBCs pass through these clots, many are damaged, which is manifested as schistocytes and helmet cells on a peripheral blood smear.
35. Answer C TTP tends to have more neurologic complications (confusion, headache, coma), while HUS has more renal complications (increased creatinine). Ingestion of E. coli O157:H7 (occasionally found in undercooked hamburger) may result in HUS. Both TTP and HUS result in schistocytes.
Questions 36-40 36. The lymphoma with which cytogenetic changes could be treated with all trans retinoic acid (ATRA)? A. t(8;21) B. t(15;17) C. inv(16)(p13;q22) D. monosomy 5 E. t(8;14)
37. Which is most likely to occur 2 weeks following a viral illness? A. TTP B. DIC C. ITP D. HUS
38. Which is most likely to be seen in geriatric medicine? A. ALL B. AML C. CML D. CLL
39. The clotting factor which is inhibited by warfarin but is also a part of the intrinsic pathway is A. II B. III C. VII D. IX E. X F.XI
40. Which of the following is the most likely inherited cause of hypercoagulability? A. protein S deficiency B. protein C deficiency C. antithrombin III deficiency D. resistance to protein S E. resistance to protein C F. resistance to antithrombin III
Answers 36-40 36. Answer B AML type M3 can occur with different genetic abnormalities (answers B, C, and D). The transversion between chromosomes 15 and 17 is treatable with ATRA, and has a favorable prognosis.
37. Answer C ITP is thought to occur from an autoimmune reaction against platelets. It can be induced after a viral illness or from drugs.
38. Answer D Chronic lymphocytic leukemia (CLL) is usually seen in patients 60 and older. By contrast, ALL is more likely to be seen in children. AML and CML tend to occur in young and middle aged adults.
39. Answer D This question requires you to know two things. First, that warfarin inhibits the production of the vitamin K dependent factors: II, V, VII, and IX. Second, you need to know that the intrinsic pathway moves through factors XII, XI, IX, VIII and then converges with the extrinsic pathway at factor X. The only factor that meets both criteria is factor IX.
40. Answer E Factor V Leiden is the most common inherited cause of hypercoagulability. It results from the change of Arg to Glu at position 506. This change prevents the cleavage (inactivation) of factor V by protein C. In other words, the individual is resistant to protein C. Factor V remains active and promotes coagulation.
Questions 41-45 41. The most important test used to distinguish anemia of chronic disease from iron deficient anemia is A. total iron binding capacity (TIBC) B. serum iron C. hematocrit D. ferritin E. peripheral blood smear
42. Which of the following peripheral blood smears would lack schistocytes? A. patient with an artificial heart valve B. patient with DIC C. patient with TTP D. patient with ITP E. patient with HUS
43. A patient appears confused upon questioning. You find the following lab values: 101.8° temperature Hgb 9.9, WBC 5,600 (normal) Plt 40,000 (low) BUN 50 (elevated) creatinine 3.1 (elevated) Which is most likely? A. TTP B. DIC C. ITP D. HUS
44. An obese man appearing cyanotic comes to the clinic for routine care for COPD. His blood reveals an increase in RBC volume, while plasma volume remains normal. Which of the following would you likely find? A. decreased serum erythropoietin B. increased serum erythropoietin C. decreased platelets and WBCs D. increased platelets and WBCs E. JAK2 mutation
45. Another patient has an increase in RBC volume with normal plasma volume. However, unlike the patient in Question 44, this patient is not hypoxic. You suspect an erythropoietin producing tumor, so you order a CT scan. Which three of following CT scan findings would be consistent with an erythropoietin producing tumor? A. lung mass B. bone mass C. liver mass D. brain mass E. kidney mass
Answers 41-45 41. Answer A In iron deficiency anemia, the patient’s iron stores (ferritin) is decreased, while the TIBC are increased. The TIBC represents the body’s attempt to retrieve iron stored in the body. The body is working very hard to use the iron it has. Anemia of chronic disease is a condition where the body is trying to sequester iron. The body is in a state of inflammation, just as if there were an infection. The body’s response to inflammation is to make less iron available for use by decreasing the TIBC. Since bacteria need iron to grow, this is beneficial for fighting off infections. However, in a patient with SLE or cancer, this response is harmful to the patient.
42. Answer D Schistocytes are the result of RBCs being traumatized. In the case of an artificial heart valve, cells are traumatized every time the valve closes. In DIC, TTP and HUS, RBCs pass through clots, which tear them apart. In ITP, there is no clot formation. Only platelets are affected.
43. Answer A TTP and HUS are very similar in presentation (thrombocytopenia with microangiopathic hemolytic anemia). However, TTP includes mental status changes, such as confusion. If this patient didn’t have confusion, HUS would be more likely.
44. Answer B This patient presents as a “blue bloater,” or an obese, cyanotic COPD patient. His chronic hypoxia (poor oxygenation) results in erythropoietin production. His high erythropoietin level is an appropriate compensation. Other patients have inappropriately high erythropoietin levels. They are usually cancer patients.
45. Answers C, D, E There are three major erythropoietin producing tumors that are worth remembering: hemartoma (liver), renal cell carcinoma, and cerebellar hemangioblastoma.
Questions 46-48 46. Using the table below, which of the following patients has sickle cell disease? 47. Using the table below, which of the following patients has pernicious anemia? 48. Using the table below, which of the following patients has a hepatoma?
Answers 46-48 46. Answer D Sickle cell anemia patients have a very high reticulocyte count because they have no problem making cells. The problem is that the cells are hemolyzed after they are made resulting in the high LDH, a non-specific marker for cell death.
47. Answer E Pernicious anemia patients cannot make new RBCs (reticulocytes) because they lack vitamin B12. They have hemolysis within the bone marrow, so the LDH increases.
48. Answer A Hepatomas can produce erythropoietin. They cause compression of the liver cells resulting in the release of alkaline phosphatase. Patient B likely has hepatitis (elevated AST), and patient C likely has Paget’s disease, a condition where bone is being constantly created and destroyed, resulting in high alkaline phosphatase levels. You’ll learn about it in musculoskeletal at the end of the year.
Fact Recall: What percent blast cells is requires to diagnose AML? 20% The spleen and liver are the primary site of hematopoiesis when? 2-7 months gestation Ratio of myeloid to erythroid precursors in bone marrow? 3:1 Von Willebrand factor carries what clotting factor? VIII
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