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.
ABSTRACT
A 41-year-old woman diagnosed with aortoarteritis since 1988 was admitted with unstable angina. She also had anemia, thrombocytopenia, aortic regurgitation and pulmonary artery hypertension. She gave a history of recurrent fetal loss and myocardial infarction, following which angioplasty to the left anterior descending artery had been done. After investigation, a diagnosis of aortoarteritis with systemic lupus erythematosus and associated antiphospholipid antibody syndrome was made. Aortoarteritis may coexist with systemic lupus erythematosus and associated antiphospholipid antibody syndrome.