Document Detail

Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura.
MedLine Citation:
PMID:  22547583     Owner:  NLM     Status:  Publisher    
Pregnancy may be complicated by a rare but life-threatening disease named thrombotic thrombocytopenic purpura (TTP). Most TTP are due to an acquired auto-immune or a hereditary severe deficiency of ADAMTS13. The interest of the differential diagnosis between acquired and hereditary TTP (Upshaw-Schulman syndrome (USS)) in an obstetrical context is poorly documented. We performed a cross-sectional analysis of the national registry of the French Reference Center for Thrombotic Microangiopathies from 2000 to 2010 to identify all women who were pregnant at their initial TTP presentation and exhibited a severe ADAMTS13 deficiency. Among 592 adulthood-onset TTP with a severe ADAMTS13 deficiency, 42 patients with a pregnancy onset-TTP were included. Surprisingly, the proportion of USS (10 patients/42 i.e. 24%, CI 13-39%) was strongly higher than in adulthood-onset TTP in general (less than 5%) and mostly related to a cluster of ADAMTS13 variants. In our USS patients, subsequent pregnancies led without prophylaxis were associated with very high TTP relapse and abortion rates while a prophylactic plasmatherapy was beneficial for both the mother and the baby. Pregnancy-onset TTP defines a specific subgroup of patients with a strong genetic background. The definitive diagnosis of hereditary TTP (USS) is crucial for the management of subsequent pregnancies.
Marie Moatti-Cohen; Céline Garrec; Martine Wolf; Pierre Boisseau; Lionel Galicier; Elie Azoulay; Alain Stepanian; Yahsou Delmas; Eric Rondeau; Stéphane Bezieau; Paul Coppo; Agnès Veyradier
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-4-30
Journal Detail:
Title:  Blood     Volume:  -     ISSN:  1528-0020     ISO Abbreviation:  -     Publication Date:  2012 Apr 
Date Detail:
Created Date:  2012-5-1     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Service d'Hematologie biologique, Hopital Antoine Beclere, Assistance Publique-Hopitaux de Paris, Clamart, France;
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