Acquired haemophilia A in the postpartum and risk of relapse in subsequent pregnancies: A systematic literature review.
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BORIS DOI
Publisher DOI
PubMed ID
33550699
Description
BACKGROUND
About 1%-5% of acquired haemophilia A cases affect mothers in the postpartum setting.
AIMS
This study delineates the characteristics of this disease, specific to the postpartum setting, notably relapse in subsequent pregnancies.
METHODS
Report of two cases and literature study (1946-2019), yielding 73 articles describing 174 cases (total 176 cases).
RESULTS
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.
CONCLUSION
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.
About 1%-5% of acquired haemophilia A cases affect mothers in the postpartum setting.
AIMS
This study delineates the characteristics of this disease, specific to the postpartum setting, notably relapse in subsequent pregnancies.
METHODS
Report of two cases and literature study (1946-2019), yielding 73 articles describing 174 cases (total 176 cases).
RESULTS
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.
CONCLUSION
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.
Date of Publication
2021-03
Publication Type
Article
Subject(s)
Keyword(s)
Factor VIII deficiency Haemophilia A acquired postpartum haemorrhage postpartum period recurrence
Language(s)
en
Contributor(s)
Dewarrat, Natacha | |
Gavillet, Mathilde | |
Naveiras, Olaia | |
Grandoni, Francesco | |
Tsakiris, Dimitrios A | |
Alberio, Lorenzo | |
Blum, Sabine |
Additional Credits
Series
Haemophilia
Publisher
Wiley
ISSN
1365-2516
Access(Rights)
restricted