Thrombotic Thrombocytopenic Purpura: A Case Report

Abstract

Thrombotic thrombocytopenic purpura is a multisystemic microvascular disorder that may be caused by an imbalance between unusually large vWF multimers and the cleaving protease ADAM TS 131 (A disintegrin and metalloproteinase with a thrombospondin type 13). This disease was first described by Moshococowitz in 1924.² It is clinically characterized by five typical syndromes: thrombocytopenia, microangiopathic hemolytic anaemia, renal impairment, fluctuating neurological abnormalities and fever. It is a rare disease and fatal in 50 to 80 per cent of cases⁵. TTP can be idiopathic or secondary. Here we are describing a case who developed fever, purpuric spot, jaundice, renal impairment and fluctuating neurological features. Initially it seems to us as a case of DIC, but later hematological evaluation reveals it as a case of TTP, as the patient fulfills the pentad criteria. Unfortunately we could not do the serum ADAM TS 13 level because of lack of facility in our country and in abroad it is costly.

Bangladesh J Medicine Jan 2015; 26 (1) : 35-38