血液疾患と輸血ジャーナル

概要

Decreased Protein C Levels in Endomyocardial Fibrosis: The Possible Significance

Prabha Nini Gupta, Kumaralingam G Balakrishnan and Kartha CC

The occurrence of frequent intracardiac thrombi in Endomyocardial fibrosis, and the embolization of these thrombi contributing to the morbidity is an established fact. Since previous studies on endomyocardial fibrosis revealed normal prothrombin time, clotting times, bleeding time, it was considered worthwhile to investigate whether an inherited deficiency of protein C contributed to thrombosis in these patients. Measurements of protein C functional levels revealed that 17/18 (94.4%) of patients tested and 4/18 of the healthy controls were functionally deficient (p<.001) while the antigenic levels of 7 patients and 3 controls were deficient. It was observed that 5.55% and 44.4% of patients had normal profiles and Type 1 deficiency respectively, while 44.4% had Type II deficiency. This association between protein C deficiency and endomyocardial fibrosis may contribute to the formation of intracardiac thrombi in these patients.