1. A 15 year old man presents with recurrent gum bleeding and intermittant petechiae, usually after trauma. There is no family history of clotting disorders. The prothrombin time, activated partial thromboplastin time (APTT), and thrombin times are all normal. The bleeding time is prolonged at 8 minutes. The Factor VIII antigen levels are 25% of normal. The most likely diagnosis is [1]:

   A. Factor VIII Deficiency
   B. Factor IX Deficiency
   C. von Willebrand Factor Deficiency
   D. Hageman Factor Deficiency
   E. Acquired Factor VIII Inhibitor

2. A 20 year old black woman with sickle cell anemia presents with extreme bone pain, fatigue, cough, tachypnea and fever to 102°C. There is no sputum production. Examination reveals diffuse pulmonary rales (crackles) and a chest radiograph confirms diffuse interstitial pulmonary edema. The alveolar-arterial oxygen (A-a) gradient is 50mmHg (normal <15mmHg). The most appropriate immediate treatment are pain medication, oxygen, incentive spirometry and [2]:

   A. Diuresis
   B. Intravenous Fluids
   C. Blood Transfusion
   D. Broad Spectrum Antibiotics
   E. Hydroxyurea

3. The same patient does well over the next several months. However, over the next year, she has six hospital admissions for painful crises and chest syndromes. Which therapy has been shown to reduce the frequency of these crises in patients with sickle cell disease [3] ?

   A. Hydroxyurea
   B. Butyrate
   C. Plasma Exchange
   D. Plasmapheresis
   E. Nocturnal Oxygen Therapy

4. A forty year old man with a 20 pack-year smoking history presents with severe pain in the right forearm. There are no systemic symptoms and no weight loss. A radiograph shows a lucency in the proximal radius. A chest radiograph shows moderate right upper lobe and mild left upper lobe interstitial infiltrates. A bone scan shows uptake only in the proximal radius corresponding to the lucency. Laboratory analysis and physical examinations are unremarkable. The most likely diagnosis is [4]:

   A. Lung Carcinoma with Bone Metastasis
   B. Testicular Carcinoma with Metastasis
   C. Pneumonia with Osteomyelitis
   D. Tuberculosis
   E. Histiocytosis

5. A 35 year old man comes in for a general evaluation after travelling to Africa for 6 months. Physical exam is unremarkable; routine laboratory tests are notable for a white blood cell count of 18K/µl with 20% eosinophils on the peripheral blood smear. There are no abnormal cell types, no blasts, no immature forms. White cell granules are normal appearing. The other blood parameters are completely normal except for a sedimentation rate of 78mm/hr. He has mild allergies and has had asthma for several years. Which is the LEAST likely diagnosis [5] ?

   A. Atopic Athma with eosinophilia
   B. Acute Myelomonocytic Leukemia with Eosinophilia (M4Eo)
   C. Churg-Strauss Syndrome
   D. Idiopathic Hypereosinophilic Syndrome
   E. Parastic Infection (intestinal)

6. An 18 year old African American woman with Hemoglobin SC disease presents with severe pain in the right pelvic area and inability to walk on her right leg. There is severe pain in the right groin which radiates through to the back. The most likely diagnosis is [6]:

   A. Pelvic Abscess
   B. Apendicitis
   C. Hip Fracture
   D. Osteonecrosis
   E. Osteoarthritis

7. All of the following can increase the platelet count EXCEPT [7]:

   A. Interleukin 11
   B. Platelet Transfusion
   C. Thrombopoietin
   D. Iron Deficiency Anemia
   E. Hemochromatosis

8. All of the following medications have been associated with a >0.5% risk of neutropenia EXCEPT [8]:

   A. Ganciclovir
   B. Carbamazapine (Tegretol®)
   C. Chloramphenicol
   D. Ticlopidine (Ticlid®)
   E. Clozapine (Clozaril®)

9. Cryoprecipitate is indicated for which ONE of the following [9]:

   A. Thrombotic Thrombocytopenic Purpura
   B. Hypofibrinogenemia
   C. Refractory Anemia with Ringed Sideroblasts
   D. Hemophilia B
   E. Chronic Pancreatitis

10. All of the following enhance platelet aggregation EXCEPT [10]:
A. Thromboxane A2
B. Adenosine Diphosphate (ADP)
C. Thrombin
D. Prostacyclin (Prostaglandin I2)
E. Exposed collagen

Answers

1. C
2. C (The chest syndrome is caused by a high content of sickled cells. Rx with blood and oxygen.)
3. A
4. E (Upper lobe infiltrates and isolated bony lesion without systemic symptoms)
5. B (Absence of abnormal cells and granules on the peripheral smear)
6. D
7. E (Iron deficiency is often associated with increased platelet count)
8. C (Chloramphenicol causes neutropania and/or aplastic anemia in 0.1% of recipients)
9. B
10. D (Prostacyclin blocks platelet aggregation)