Korean J Obstet Gynecol.  2010 May;53(5):434-442. 10.5468/kjog.2010.53.5.434.

Thrombotic thrombocytopenic purpura in three pregnancies

Affiliations
  • 1Department 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.

Keyword

Thrombotic thrombocytopenic purpura; HELLP Syndrome; Pre-Eclampsia; ADAMTS-13; Pregnancy complications; Hematologic/pathology/therapy

MeSH Terms

Anemia, Hemolytic
Central Nervous System
Diagnostic Tests, Routine
Female
Fever
HELLP Syndrome
Humans
Plasma Exchange
Plasmapheresis
Pre-Eclampsia
Pregnancy
Pregnancy Complications
Prognosis
Purpura, Thrombocytopenic, Idiopathic
Purpura, Thrombotic Thrombocytopenic
Thrombocytopenia
Thrombotic Microangiopathies
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