A Case of Thrombotic Thrombocytopenic Purpura with Exacerbation after Incomplete Remission of Therapeutic Plasma Exchange.
10.17945/kjbt.2017.28.3.298
- Author:
Yong Hak SOHN
1
;
Chunhwa IHM
;
Eun Hye CHOI
;
Sang Kwang LEE
Author Information
1. Department of Laboratory Medicine, Eulji University Hospital, Daejeon, Korea. haneul@eulji.ac.kr
- Publication Type:Case Report
- Keywords:
Thrombotic thrombocytopenic purpura;
ADAMTS13 activity;
ADAMTS13 inhibitor;
Plasma exchange;
Plasmapheresis
- MeSH:
Adult;
Humans;
Male;
Methods;
Mortality;
Plasma Exchange*;
Plasma*;
Plasmapheresis;
Purpura, Thrombotic Thrombocytopenic*;
Retreatment;
Survival Rate;
von Willebrand Factor
- From:Korean Journal of Blood Transfusion
2017;28(3):298-303
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder with a mortality rate of over 90% without prompt treatment. It is caused by congenital, idiopathic, or secondary diseases; idiopathic TTP is mainly associated with deficiency of ADAMTS13, a von Willebrand factor cleaving protease or ADAMTS13 inhibitors. The long-term survival rate of TTP has improved since the introduction of therapeutic plasma exchange (TPE), and the therapeutic aims have also been established. However, deciding on the end-point and appropriate treatment method requires careful assessment of clinical conditions of patients. The present study reports a case of a 33-year-old male patient with reduced ADAMTS13 activity and ADAMTS13 inhibitor, who developed symptoms after an early termination of TPE with improved symptoms, which finally improved with retreatment and additionally corticosteroid. We report our case with relevant literature review on TPE in TTP with this case.
