Chapter·Internal MedicineHematology (anemias, clotting disorders)

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1

A previously healthy 17-year-old boy is brought to the emergency department by his mother for further evaluation after elective removal of his wisdom teeth. During the procedure, the patient had persistent bleeding from the teeth's surrounding gums. Multiple gauze packs were applied with minimal effect. The patient has a history of easy bruising. The mother says her brother had similar problems when his wisdom teeth were removed, and that he also has a history of easy bruising and joint swelling. The patient takes no medications. His temperature is 37°C (98.6°F), pulse is 90/min, and blood pressure is 108/74 mm Hg. Laboratory studies show: Hematocrit 35% Leukocyte count 8,500/mm3 Platelet count 160,000/mm3 Prothrombin time 15 sec Partial thromboplastin time 60 sec Bleeding time 6 min Fibrin split products negative Serum Urea nitrogen 20 mg/dL Creatinine 1.0 mg/dL Bilirubin Total 1.0 mg/dL Direct 0.5 mg/dL Lactate dehydrogenase 90 U/L Peripheral blood smear shows normal-sized platelets. Which of the following is the most likely diagnosis?

AHemophilia

BBernard-Soulier syndrome

CGlanzmann thrombasthenia

DVon Willebrand disease

EImmune thrombocytopenia

2

A 34-year-old, previously healthy woman is admitted to the hospital with abdominal pain and bloody diarrhea. She reports consuming undercooked beef a day before the onset of her symptoms. Her medical history is unremarkable. Vital signs include: blood pressure 100/70 mm Hg, pulse rate 70/min, respiratory rate 16/min, and temperature 36.6℃ (97.9℉). Physical examination shows paleness, face and leg edema, and abdominal tenderness in the lower right quadrant. Laboratory investigation shows the following findings: Erythrocytes 3 x 106/mm3 Hemoglobin 9.4 g/dL Hematocrit 0.45 (45%) Corrected reticulocyte count 5.5% Platelet count 18,000/mm3 Leukocytes 11,750/mm3 Total bilirubin 2.33 mg/dL (39.8 µmol/L) Direct bilirubin 0.2 mg/dL (3.4 µmol/L) Serum creatinine 4.5 mg/dL (397.8 µmol/L) Blood urea nitrogen 35.4 mg/dL (12.6 mmol/L) E. coli O157: H7 was identified in the patient’s stool. Which toxin is likely responsible for her symptoms?

AErythrogenic toxin

BShiga toxin

CEnterotoxin type B

Dα-hemolysin

EExotoxin A

3

A 66-year-old woman presents to the emergency department complaining of palpitations. She says that she has been experiencing palpitations and lightheadedness for the past 6 months, but before this morning the episodes usually resolved on their own. The patient's medical history is significant for a transient ischemia attack 2 months ago, hypertension, and diabetes. She takes aspirin, metformin, and lisinopril. She states her grandfather died of a stroke, and her mom has a "blood disorder." An electrocardiogram is obtained that shows an irregularly irregular rhythm with rapid ventricular response, consistent with atrial fibrillation. She is given intravenous metoprolol, which resolves her symptoms. In addition to starting a beta-blocker for long-term management, the patient meets criteria for anticoagulation. Both unfractionated heparin and warfarin are started. Five days later, the patient begins complaining of pain and swelling of her left lower extremity. A Doppler ultrasound reveals thrombosis in her left popliteal and tibial veins. A complete blood count is obtained that shows a decrease in platelet count from 245,000/mm^3 to 90,000/mm^3. Coagulation studies are shown below: Prothrombin time (PT): 15 seconds Partial thromboplastin time (PTT): 37 seconds Bleeding time: 14 minutes Which of the following is the most likely diagnosis?

AThrombotic thrombocytopenic purpura

BType I heparin-induced thrombocytopenia

CWarfarin toxicity

DIdiopathic thrombocytopenia purpura

EType II heparin-induced thrombocytopenia

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