Protein C deficiency leading to pulmonary thromboembolism in a patient with hereditary spherocytosis.

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2006-11-06
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Abstract
Hereditary protein C deficiency results in a hypercoagulable state that can manifest itself as venous thrombosis and pulmonary embolism. The prevalence of this condition, even among patients with familial thrombosis, is quite low. We report a case of protein C deficiency presenting as massive pulmonary thromboembolism in a patient with hereditary spherocytosis, an uncommon hemolytic disorder not usually associated with increased thrombotic risk. A review of the literature revealed only a few cases of thrombosis associated with hereditary spherocytosis, and none of them had protein C deficiency. This makes the present case the first of its kind to be reported.
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Bhargava K, Chandra N, Omar AK, Mathur A. Protein C deficiency leading to pulmonary thromboembolism in a patient with hereditary spherocytosis. Indian Heart Journal. 2006 Nov-Dec; 58(6): 444-6