Chapter·Internal MedicineHematology (anemias, clotting disorders)

Hypercoagulable statesDownloads

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1

A 21-year-old woman comes to the physician because of a 1-day history of right leg pain. The pain is worse while walking and improves when resting. Eight months ago, she was diagnosed with a pulmonary embolism and was started on warfarin. Anticoagulant therapy was discontinued two months ago. Her mother had systemic lupus erythematosus. On examination, her right calf is diffusely erythematous, swollen, and tender. Cardiopulmonary examination shows no abnormalities. On duplex ultrasonography, the right popliteal vein is not compressible. Laboratory studies show an elevated serum level of D-dimer and insensitivity to activated protein C. Further evaluation of this patient is most likely to show which of the following?

ADeficiency of protein C

BProtein S deficiency

CElevated coagulation factor VIII levels

DMutation of coagulation factor V

EMutation of prothrombin

2

A 3-week-old boy is brought to the pediatrician by his parents for a circumcision. The circumcision was uncomplicated; however, after a few hours, the diaper contained blood, and the bleeding has not subsided. A complete blood count was ordered, which was significant for a platelet count of 70,000/mm3. On peripheral blood smear, the following was noted (figure A). The prothrombin time was 12 seconds, partial thromboplastin time was 32 seconds, and bleeding time was 13 minutes. On platelet aggregation studies, there was no response with ristocetin. This result was not corrected with the addition of normal plasma. There was a normal aggregation response with the addition of ADP. Which of the following is most likely true of this patient's underlying disease?

AProtein C resistance

BDecreased GpIb

CResponsive to desmopressin

DAdding epinephrine would not lead to platelet aggregation

EDecreased GpIIb/IIIa

3

A 29-year-old woman comes to the office with her husband because she has had 4 spontaneous abortions. Regarding her medical history, she was diagnosed with systemic lupus erythematosus 9 years ago, had a stroke 3 years ago, and was diagnosed with deep vein thrombosis in the same year. She has no relevant family history. Her vital signs include: heart rate 78/min, respiratory rate 14/min, temperature 37.5°C (99.5°F), and blood pressure 120/85 mm Hg. The physical examination is unremarkable. The complete blood count results are as follows: Hemoglobin 12.9 g/dL Hematocrit 40% Leukocyte count 8,500/mm3 Neutrophils 55% Bands 2% Eosinophils 1% Basophils 0% Lymphocytes 29% Monocytes 2% Platelet count 422,000/mm3 Her coagulation test results are as follows: Partial thromboplastin time (activated) 50.9 s Prothrombin time 13.0 s A VDRL test is done, and the result is positive. Mixing studies are performed, and they fail to correct aPTT. What is the most likely cause in this patient?

AAntithrombin deficiency

BProtein S deficiency

CProtein C deficiency

DAntiphospholipid syndrome

EFactor V Leiden mutation

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