A Patient withThrombotic Thrombocytopenic Purpura Who Completely Fulfills All Pentad: A Case Report

Abstract

Abstract:

Thrombotic thrombocytopenic purpura (TTP) is a rare but serious disease that is characterized by the microangiopathic hemolytic anemia (MAHA), thrombocytopenia, neurologic abnormalities, kidney impairment and fever, the so-called pentad. So far it has been rarely recognized among Thai patients. And herein, we reported one patient who completely fulfilled all pentad of TTP which was confirmed by the severe deficiency of ADAMTS13 activity. He was a 70-year-old Thai man who suddenly developed confusion and disorientation within a day. Besides confusion, the physical examination revealed fever and frank pallor. The blood tests included Hb 7.4 g%, WBC 7,700/mm³, platelet 17,000/mm³, with the MAHA picture on the peripheral blood smear, reticulocyte 4.1%, creatinine 1.3 mg%, GFR 55 ml/min/1.73 m², and the ADAMTS13 factor activity was less than 3%.The computerized tomography of the brain revealed unremarkable study. He was diagnosed as having TTP therefore he was treated with the daily plasmapheres is. The complete fulfillment of all pentad of TTP is not commonly found in most cases who always present with only MAHA, thrombocytopenia with ADAMTS13 deficiency. He dramatically responded to therapy with the complete recovery of all pentad. The plasmapheresis is the highly effective method of treatment for TTP probably because it could remove the large and ultra-large von Willebrand Factor, remove the ADAMTS13 inhibitors and proteolytic inactivators, and replenish ADAMTS13 that is severely lacked in most cases of TTP.