Thrombotic thrombocytopenic purpura in three pregnancies.

Yoon, Won Sik; Lee, Jeong Won; Kim, Yeon Hee; Ahn, Hyun Young; Shin, Jong Chul

doi:2010.53.5.434

Obstet Gynecol Sci > Volume 53(5); 2010 > Article

Case Report

Korean Journal of Obstetrics & Gynecology 2010;53(5):434-442.
DOI: https://doi.org/10.5468/kjog.2010.53.5.434 Published online May 1, 2010.

Thrombotic thrombocytopenic purpura in three pregnancies.

Won Sik Yoon, Jeong Won Lee, Yeon Hee Kim, Hyun Young Ahn, Jong Chul Shin

Department of Obstetrics and Gynecology, The Catholic University of Korea School of Medicine, Seoul, Korea. sono@catholic.ac.kr

Abstract

Thromobotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS), characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, central nervous system abnormalities, and renal dysfunction, is severe multisystem disorder. TTP-HUS occurs predominantly in the reproductive aged-women, associated with poor prognosis. Although the morbidity and mortality have been significantly decreased by using plasma exchange therapy, refractory TTP-HUS remains a tremendous problem. It is crucial to differentiate other microangiopathic hemolytic anemia disease with a confusing presentation and to perform the immediate plasmapheresis. We have experienced three cases, which were initially diagnosed as HELLP syndrome or immune thrombocytopenic purpura. Despite of aggressive plasmapheresis, two women died. We present these cases with a review of the literature on pregnancy-associated thrombotic microangiopathy, including ADAMTS-13 activity assay as a new diagnostic test.

Key Words: Thrombotic thrombocytopenic purpura, HELLP Syndrome, Pre-Eclampsia, ADAMTS-13, Pregnancy complications, Hematologic/pathology/therapy