Korean J Blood Transfus.  2017 Dec;28(3):298-303. 10.17945/kjbt.2017.28.3.298.

A Case of Thrombotic Thrombocytopenic Purpura with Exacerbation after Incomplete Remission of Therapeutic Plasma Exchange

• Sohn YH
• Ihm C
• Choi EH
• Lee SK

• KMID: 2401748
• DOI: http://doi.org/10.17945/kjbt.2017.28.3.298

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.

Keyword

Thrombotic thrombocytopenic purpura; ADAMTS13 activity; ADAMTS13 inhibitor; Plasma exchange; Plasmapheresis

MeSH Terms

Adult
Humans
Male
Methods
Mortality
Plasma Exchange*
Plasma*
Plasmapheresis
Purpura, Thrombotic Thrombocytopenic*
Retreatment
Survival Rate
von Willebrand Factor
von Willebrand Factor