1. Hemolytic anemia is NOT associated with which of the following medications [1]:

   A. Procainamide
   B. Alpha-methyldopa
   C. Doxycyline
   D. Penicillin
   E. Sulfasalazine

2. A 35 year old woman presents to the emergency room with two days of lower extremity bruising, fatigue, and mild confusion. She had been well previously and takes only oral contraceptive and multivitamin pills. She does not smoke, drinks alcohol only one to two times per week socially, and uses no recreational drugs. Except for two urinary tract infections in the past, she has no signficiant medical history.The patient noted bruising in her right calf two days prior to presentation. In addition, there was some mild gum bleeding. Menstruations were light and normal two weeks ago. Over the past 24 hours she has noted increased confusion, usually about where she is and where she is going. There may have been mild fevers which were intermittant, and were without chills or night sweats. Mild headaches and nausea has recently occurred. There has been no weight loss, chest or abdominal pain, and her bowel movements have been normal.Physical examination reveals a normal appearing young woman in mild distress. Her blood pressure is 145/95, pulse 110, respirations 24, and temperature 100.3°F. Head and neck exam are normal except for mild gum bleeding. There are petechiae on the forearms, chest, and small ecchymoses on the lower extremities with some petechiae. Chest is clear. The cardiac exam is normal except for regular tachycardia and a I/VI systolic ejection murmer at the base. The abdomen is soft with very active bowel sounds, nontender, and no masses or organomegaly.Initial laboratory data show a white count of 12K/µl with a normal differential, HCT 29%, Platelets 39K/µl, MCV 103fl, PT/PTT pending, sodium 139 mM, potassium 6.1 mM, chloride 104, bicarbonate 18, BUN 50, creatinine 2.8, glucose 110, liver function tests pending.
3. What is the most likely cause of the patient's symptoms, signs, and blood tests [2] ?

   A. Microangiopathic hemolytic anemia
   B. Sepsis
   C. Pancreatitis
   D. Rickettsial Infection
   E. Toxic Shock Syndrome

4. The PT and PTT results are reported as normal. Which of the following is less likely to be present [3] ?

   A. Hemolytic Uremic Syndrome (HUS)
   B. Disseminated Intravascular Coagulopathy (DIC)
   C. Thrombotic Thrombocytopenic Purpura (TTP)
   D. Microangiopathic hemolytic anemia
   E. Toxic Shock Syndrome

5. Which of the following infections are most often associated with the above syndrome [4] ?

   A. Toxin producing Staphylococcus aureus
   B. Toxigenic Escherichia coli
   C. Group A ß-hemolytic streptococci
   D. Pseudomonas aurugenosa
   E. Streptococcus pyogenes

6. Which treatments below has been shown to be most effective in this syndrome [5] ?

   A. Broad spectrum antibiotics
   B. Plasma Infusions alone
   C. Intensive monitoring, fluid infusions, and low dose ("renal range") dopamine
   D. Plasma Exchange Therapy
   E. Platelet transfusions, Fresh Frozen Plasma, and Intravenous Globulin (IVIg)

7. Which of the following has NOT been effective in the treatment of autoimmune (idiopathic)

• Which of the following is NOT associated with a hyper-coagulable state [7] ?

  A. Homocysteinuria
  B. Antiphospholipid antibodies
  C. Elevated Serum Triglycerides
  D. Polyarteritis Nodosa
  E. Hyperfibrinogenemia

• Which of the following is characterized as a Megaloblastic Anemia [8] ?

  A. Hemolytic Anemia with Elevated Reticulocyte Count (MCV 102 fL)
  B. Chronic Liver Disease (MCV 101 fL)
  C. Refractory Anemia with Ringed Sideroblasts (RARS; MCV 104 fL)
  D. Copper Deficiency (MCV 100 fL)
  E. Hereditary Elliptocytosis (MCV 99 fL)

• A 30 year old healthy, nonsmoking woman on no medications develops a deep vein thrombosis in her left leg during a cross-country bus ride. She is treated with heparin for 5 days and warfarin (coumadin®) is started. She is discharged from the hospital on coumadin with an INR (international normalization ratio) of 2.1. Two weeks later she presents with severe pain in her right foot. Examination reveals an infarction of the third digit with severe pain, purpura, and erythema. Her INR is 2.2 and she has been compliant with her medication. The patient's sister was treated for a minor stroke at the age of 28 years old. The most likely diagnosis is [9]:

  A. Warfarin induced skin necrosis
  B. Protein C deficiency
  C. Factor IX deficiency
  D. Paroxysmal Nocturnal Hemoglobinuria
  E. Homocysteinuria

• A 45 year old man presents with right sided paralysis and expressive aphasia. A computerized tomographic scan of the head without contrast shows no hemorrhage. The patient is treated with one aspirin and moderate dose of heparin with complete resolution of symptoms after 10 hours. The heparin is continued for several days. Laboratory evaluation reveals a normal viscosity, a prothrombin time of 11.2 seconds (control 11.5 seconds), activated partial thromboplastin time (APTT) of 45 seconds (control 28 seconds), anticardiolipin antibodies in the normal range, and a prolonged dilute russel viper venom time. Which of the following tests is likely to confirm the diagnosis [10] ?
A. Serum Mixing Study (APTT measurement)
B. Factor IX Inhibitor Level
C. Factor VIII Level
D. Rapid Plasma Reagin Test (RPR)
E. Bleeding Time

Answers

1. C
2. A
3. B
4. B
5. D
6. E
7. C
8. C
9. B
10. A (An inhibitor of coagulation is present; mixing with normal serum will not correct APTT)