Acquired haemophilia A in the postpartum and risk of relapse in subsequent pregnancies: A systematic literature review.

Details

Serval ID
serval:BIB_C7857B4FC41A
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Acquired haemophilia A in the postpartum and risk of relapse in subsequent pregnancies: A systematic literature review.
Journal
Haemophilia
Author(s)
Dewarrat N., Gavillet M., Angelillo-Scherrer A., Naveiras O., Grandoni F., Tsakiris D.A., Alberio L., Blum S.
ISSN
1365-2516 (Electronic)
ISSN-L
1351-8216
Publication state
Published
Issued date
03/2021
Peer-reviewed
Oui
Volume
27
Number
2
Pages
199-210
Language
english
Notes
Publication types: Journal Article ; Review
Publication Status: ppublish
Abstract
About 1%-5% of acquired haemophilia A cases affect mothers in the postpartum setting.
This study delineates the characteristics of this disease, specific to the postpartum setting, notably relapse in subsequent pregnancies.
Report of two cases and literature study (1946-2019), yielding 73 articles describing 174 cases (total 176 cases).
Patients were aged 29.9 years (17-41) and 69% primigravidae. Diagnosis was made at a median of 60 days after delivery (range 0-308). Bleeding types were obstetrical (43.4%), cutaneous (41.3%), and muscular (36.7%). In >90% of the cases, FVIII at diagnosis was <1% (range 0%-8%). FVIII inhibitor was documented in 75.4% cases (median titre of 20 BU/ml, range 1-621). Haemostatic treatment was necessary in 57.1% using fresh frozen plasma (16%), factor concentrate (27.6%) and/or bypassing agents (37.4%). Immunosuppressive treatment was administered in 90.8%, mostly steroids (85.3%), alone or combined with immunosuppressants (27%). Rituximab was used mostly as a second line treatment. Only 24 patients (13.6%) had documented subsequent pregnancies and 6 (22.2%) suffered haemophilia recurrence during pregnancy.
This study allows better definition of: (1) clinical and laboratory characteristics of postpartum acquired haemophilia, (2) response to therapy, and (3) the risk of relapse for subsequent pregnancies.
Keywords
Factor VIII deficiency, Haemophilia A, acquired, postpartum haemorrhage, postpartum period, recurrence
Pubmed
Web of science
Create date
22/02/2021 15:36
Last modification date
05/05/2021 6:36
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