1. You are asked to evaluate a 34-year-old female for abnormal coagulation tests. She gives a history of bleeding after tooth extraction and tonsillectomy, as well as heavy menstrual periods. Her mother and sister also have histories of excessive bleeding. The patient's CBC is normal; the PT is 12 sec and the PTT is 54 sec.The least likely diagnosis is:
A. Factor IX deficiency
B. Lupus anticoagulant
C. von Willebrand's disease
D. Factor XI deficiency
E. Factor VIII deficiency
A. Factor IX deficiency
B. Lupus anticoagulant
C. von Willebrand's disease
D. Factor XI deficiency
E. Factor VIII deficiency
2. In which disorder is thrombocytopenia least likely to be seen?
A. Aplastic anemia
B. Splenomegaly
C. Myeloproliferative disorder
D. Disseminated intravascular coagulation
E. Bernard-Soulier syndrome
A. Aplastic anemia
B. Splenomegaly
C. Myeloproliferative disorder
D. Disseminated intravascular coagulation
E. Bernard-Soulier syndrome
3. A patient is diagnosed with Factor V Leiden. Which statement is not correct about this disorder?
A. Factor V Leiden resists degradation by activated protein C.
B. This is a genetic disease.
C. Protein C deficiency is more common than Factor V Leiden.
D. Factor V Leiden is an autosomal dominant disorder.
E. Factor V Leiden is a thrombotic disorder.
A. Factor V Leiden resists degradation by activated protein C.
B. This is a genetic disease.
C. Protein C deficiency is more common than Factor V Leiden.
D. Factor V Leiden is an autosomal dominant disorder.
E. Factor V Leiden is a thrombotic disorder.
4. What tissue serves to provide the majority of blood cells in the mid-gestation fetus?
A. Yolk sac
B. Bone marrow
C. Liver
D. Spleen
E. Thymus
A. Yolk sac
B. Bone marrow
C. Liver
D. Spleen
E. Thymus
5. A 60-year-old man presents with aplastic anemia. What is the most appropriate management plan?
A. HLA typing of siblings in preparation for bone marrow transplant
B. Growth factor support (G-CSF and erythropoietin)
C. Anti-thymocyte globulin (ATG) plus cyclosporin
D. Low molecular weight heparin
E. Bone marrow transplantation from a matched, unrelated donor
A. HLA typing of siblings in preparation for bone marrow transplant
B. Growth factor support (G-CSF and erythropoietin)
C. Anti-thymocyte globulin (ATG) plus cyclosporin
D. Low molecular weight heparin
E. Bone marrow transplantation from a matched, unrelated donor
6. A 50-year-old lawyer is seen in your clinic for an insurance physical exam. He is feeling well overall but says that over the past 2 months he has been suffering from increased fatigue and has lost 10 pounds. He is putting in long hours at work because of an important medical malpractice case he is involved in. He takes no medications. His past medical history is significant only for a splenectomy following a gunshot wound he sustained in Vietnam. You find no significant abnormality on his physical exam except for a surgical scar on his abdomen. His laboratory exam reveals a white blood cell count of 15,000/µl, a platelet count of 550,000/µl, and a hematocrit of 52%. You review the patient's blood film and notice a leukocytosis with Howell-Jolly bodies in his red blood cells. At this point you should:
A. Do a bone marrow biopsy to rule out leukemia, particularly with a history of fatigue and weight loss.
B. Reassure the patient that his leukocytosis is expected and is secondary to his splenectomy.
C. Since the patient is a malpractice lawyer and you are afraid of being sued, do a bone marrow biopsy to be sure that nothing is wrong.
D. Start the patient on antibiotics just in case he may have an infection.
E. Obtain a stool sample to determine if he has parasites.
A. Do a bone marrow biopsy to rule out leukemia, particularly with a history of fatigue and weight loss.
B. Reassure the patient that his leukocytosis is expected and is secondary to his splenectomy.
C. Since the patient is a malpractice lawyer and you are afraid of being sued, do a bone marrow biopsy to be sure that nothing is wrong.
D. Start the patient on antibiotics just in case he may have an infection.
E. Obtain a stool sample to determine if he has parasites.
7. You are asked to evaluate a 69 year old woman with diffuse adenopathy and guaiac positive stools. She has been fatigued and notes night sweats about two times per week for the past three months. She has already had a biopsy of one of the lymph nodes, and it revealed a diffuse pattern with large B cells that were monoclonal. What is your next step?
A. Staging evaluation including CT scans of the chest, abdomen and pelvis, and bone marrow aspirate and biopsy.
B. Careful physical examination with attention to vital signs (in particular blood pressure and pulse), lymph node groups and rectal exam.
C. Immediate treatment with CHOP chemotherapy.
D. Referral for bone marrow transplant.
A. Staging evaluation including CT scans of the chest, abdomen and pelvis, and bone marrow aspirate and biopsy.
B. Careful physical examination with attention to vital signs (in particular blood pressure and pulse), lymph node groups and rectal exam.
C. Immediate treatment with CHOP chemotherapy.
D. Referral for bone marrow transplant.
8. A 48-year-old Caucasian man develops fatigue, weakness, and early satiety. The physical exam reveals pallor and splenomegaly. The spleen extends 6 cm below the left costal margin. Laboratory tests reveal a hemoglobin concentration of 9.0 g/dL, hematocrit 27%, MCV 88 fL, platelet count 600,000/µL, white blood cell count 210,000/µL. A leukocyte differential count reveals 50% segmented neutrophils, 8% band forms, 4% metamyelocytes, 4% myelocytes, 3% promyelocytes, 1% blasts, 12% monocytes, 8% basophils, 4% eosinophils, and 6% lymphocytes. A marrow aspiration and biopsy reveals a hypercellular marrow with a myeloid:erythroid ratio of 9:1. Which of the following tests would most likely establish a specific diagnosis?
A. A leukocyte alkaline phosphatase (LAP) score.
B. A thrombopoietin assay.
C. Special stains of the marrow to detect deposition of collagen.
D. A bone marrow karyotype (cytogenetic study).
E. An erythropoietin assay.
A. A leukocyte alkaline phosphatase (LAP) score.
B. A thrombopoietin assay.
C. Special stains of the marrow to detect deposition of collagen.
D. A bone marrow karyotype (cytogenetic study).
E. An erythropoietin assay.
9. A 70-year-old Caucasian woman, previously healthy, develops fatigue, weakness, and exertional dyspnea. The physical examination reveals only pallor. An anemia is detected with a hemoglobin concentration of 7.0 g/dL and an hematocrit of 21% with an MCV of 105 fL. The white blood cell count is 8,000/µL and the platelet count is 510,000/µL. The bone marrow is hypercellular. Red cell precursors are reduced in number. Megakaryocytes are smaller than normal and most have a single, round nucleus. Which of the following is most likely to be found?
A. Mitochondrial iron deposits in erythroid precursors.
B. Translocation of DNA from chromosome 9q to chromosome 22.
C. An abnormal first stage Schilling test, but a normal second stage.
D. Deletion of the long arm (q) of chromosome 5.
E. Blasts containing Auer rods in the blood and marrow.
A. Mitochondrial iron deposits in erythroid precursors.
B. Translocation of DNA from chromosome 9q to chromosome 22.
C. An abnormal first stage Schilling test, but a normal second stage.
D. Deletion of the long arm (q) of chromosome 5.
E. Blasts containing Auer rods in the blood and marrow.
10. A healthy 19-year-old Caucasian woman develops right upper quadrant abdominal pain. A physical exam reveals right upper quadrant tenderness and just a "hint" of scleral icterus. The spleen tip is just palpable. Laboratory tests reveal a hemoglobin concentration of 12 g/dL and an hematocrit of 36%. The MCV is 90 fL. The reticulocyte count is 5.2%. The total bilirubin is 2.3 mg/dL with an indirect fraction of 2.0 mg/dL. An ultrasound examination reveals several large gallstones. A laparoscopic cholecystectomy is performed. At the time of discharge, she is given her gallstones because they are unusual in appearance. Instead of the usual yellow-tan color, they are deeply pigmented. Which one of the following laboratory tests is likely to be abnormal?
A. Assay of glucose-6-phosphate dehydrogenase activity in an erythrocyte lysate.
B. Mutational analysis of the pyruvate kinase gene.
C. Mutational analysis of the (-spectrin gene.
D. Mutational analysis of the bilirubin-glucoronyltransferase gene.
E. A Heinz body preparation.
A. Assay of glucose-6-phosphate dehydrogenase activity in an erythrocyte lysate.
B. Mutational analysis of the pyruvate kinase gene.
C. Mutational analysis of the (-spectrin gene.
D. Mutational analysis of the bilirubin-glucoronyltransferase gene.
E. A Heinz body preparation.
11. The methylfolate "trap" hypothesis is based on which one of the following findings?
A. The intracellular formation of folate polyglutamates is a B12-dependent reaction.
B. The conversion of methylmalonyl co-enzyme A to succinyl CoA is catalyzed by a B12-dependent reaction.
C. The synthesis of methionine from homocysteine is a B12-dependent reaction.
D. Gastrointestinal absorption of folic acid is a B12-dependent reaction.
E. The incorporation of deoxythymidine monophosphate into DNA is a B12-dependent reaction.
A. The intracellular formation of folate polyglutamates is a B12-dependent reaction.
B. The conversion of methylmalonyl co-enzyme A to succinyl CoA is catalyzed by a B12-dependent reaction.
C. The synthesis of methionine from homocysteine is a B12-dependent reaction.
D. Gastrointestinal absorption of folic acid is a B12-dependent reaction.
E. The incorporation of deoxythymidine monophosphate into DNA is a B12-dependent reaction.
12. Match the clinical findings with the disease most likely associated with the finding: Petechiae
A. Protein C deficiency
B. ITP
C. Miscarriage
D. Hemophilia A
E. Liver disease
F. Vitamin K deficiency
A. Protein C deficiency
B. ITP
C. Miscarriage
D. Hemophilia A
E. Liver disease
F. Vitamin K deficiency
13. Match the clinical findings with the disease most likely associated with the finding: Hemarthrosis
A. Protein C deficiency
B. ITP
C. Miscarriage
D. Hemophilia A
E. Liver disease
F. Vitamin K deficiency
A. Protein C deficiency
B. ITP
C. Miscarriage
D. Hemophilia A
E. Liver disease
F. Vitamin K deficiency
14. Match the clinical findings with the disease most likely associated with the finding: Lupus anticoagulant
A. Protein C deficiency
B. ITP
C. Miscarriage
D. Hemophilia A
E. Liver disease
F. Vitamin K deficiency
A. Protein C deficiency
B. ITP
C. Miscarriage
D. Hemophilia A
E. Liver disease
F. Vitamin K deficiency
15. Match the clinical findings with the disease most likely associated with the finding: Pulmonary embolism
A. Protein C deficiency
B. ITP
C. Miscarriage
D. Hemophilia A
E. Liver disease
F. Vitamin K deficiency
A. Protein C deficiency
B. ITP
C. Miscarriage
D. Hemophilia A
E. Liver disease
F. Vitamin K deficiency
16. A 40-year-old Vietnamese male is seen in a health clinic for untreated tuberculosis. His hematocrit is 34% (normal 39-49%). The MCV is 78 fl (normal 83-97 fl). The serum iron is low, and the TIBC is normal. The ferritin value is elevated. Why is the serum iron low in the patient?
A. He is iron-deficient from occult G.I. blood loss.
B. He has hemolytic anemia.
C. Iron release from macrophages is impaired.
D. Vitamin B12 absorption is not normal.
E. He has folate deficiency due to malnutrition.
A. He is iron-deficient from occult G.I. blood loss.
B. He has hemolytic anemia.
C. Iron release from macrophages is impaired.
D. Vitamin B12 absorption is not normal.
E. He has folate deficiency due to malnutrition.
17. Which of the following anemias is usually not macrocytic?
A. Hemolytic anemia.
B. Liver disease.
C. Folate deficiency.
D. Anemia of chronic disease.
E. Myelodysplasia.
A. Hemolytic anemia.
B. Liver disease.
C. Folate deficiency.
D. Anemia of chronic disease.
E. Myelodysplasia.
18. A 59-year-old woman complains of headaches and visual difficulties. Her examination shows a ruddy complexion and a spleen palpable 3 cm below the left costal margin. Her complete blood count shows:
- Hematocrit: 59% (normal 39-49%)
- WBC: 12,300/Fl (normal 4,000-9,000/Fl)
- Platelets: 650,000/Fl (normal 140,000-440,000/Fl)
Her arterial oxygen saturation is 95% on room air. You recommend which of the following:
A. Chemotherapy with fludarabine and prednisone.
B. Phlebotomy.
C. Observation.
D. Allogeneic bone marrow transplantation.
E. Inhaled oxygen therapy @ 2 L/min.
19. Which one of the following statements about classification of anemias is NOT correct?
A. Increased indirect bilirubin, elevated LDH, and an increased reticulocyte count is characteristic of both untreated megaloblastic anemia and autoimmune hemolytic anemia.
B. Most sideroblastic anemias are macrocytic.
C. Anemia of chronic disease may be either normocytic or microcytic.
D. Thalassemia can be categorized either as a microcytic anemia or as a hemolytic anemia.
E. Metastatic breast cancer, myeloma, and myelofibrosis are all causes of myelophthisic anemia.
A. Increased indirect bilirubin, elevated LDH, and an increased reticulocyte count is characteristic of both untreated megaloblastic anemia and autoimmune hemolytic anemia.
B. Most sideroblastic anemias are macrocytic.
C. Anemia of chronic disease may be either normocytic or microcytic.
D. Thalassemia can be categorized either as a microcytic anemia or as a hemolytic anemia.
E. Metastatic breast cancer, myeloma, and myelofibrosis are all causes of myelophthisic anemia.
20. Hemoglobin and hematocrit values are usually, but not always, accurate indicators of total body red cell mass. Under what circumstances would the hematocrit or hemoglobin NOT correlate with red cell mass?
A. Megaloblastic anemia.
B. Hemoglobin of altered oxygen affinity.
C. Acute blood loss.
D. Severe iron deficiency.
E. Hereditary spherocytosis.
A. Megaloblastic anemia.
B. Hemoglobin of altered oxygen affinity.
C. Acute blood loss.
D. Severe iron deficiency.
E. Hereditary spherocytosis.
21. A 23-year-old man has had three episodes of venous thrombosis. An appropriate laboratory evaluation should include all of the following except:
A. Protein C.
B. Protein S.
C. Antithrombin III.
D. Factor V Leiden.
E. Alpha2-macroglobulin
Answers
1.B
2.C
3.C
4.C
5.C
6.B
7.B
8.D
9.D
10.C
11.C
12.B
13.D
14.C
15.A
16.C
17.D
18.B
19.A
20.C
21.E
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