Acquired haemophilia A in the postpartum and risk of relapse in subsequent pregnancies: A systematic literature review.
Factor VIII deficiency
Haemophilia A
acquired
postpartum haemorrhage
postpartum period
recurrence
Journal
Haemophilia : the official journal of the World Federation of Hemophilia
ISSN: 1365-2516
Titre abrégé: Haemophilia
Pays: England
ID NLM: 9442916
Informations de publication
Date de publication:
Mar 2021
Mar 2021
Historique:
revised:
05
12
2020
received:
10
06
2020
accepted:
09
12
2020
pubmed:
8
2
2021
medline:
25
9
2021
entrez:
7
2
2021
Statut:
ppublish
Résumé
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.
Sections du résumé
BACKGROUND
BACKGROUND
About 1%-5% of acquired haemophilia A cases affect mothers in the postpartum setting.
AIMS
OBJECTIVE
This study delineates the characteristics of this disease, specific to the postpartum setting, notably relapse in subsequent pregnancies.
METHODS
METHODS
Report of two cases and literature study (1946-2019), yielding 73 articles describing 174 cases (total 176 cases).
RESULTS
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
CONCLUSIONS
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.
Substances chimiques
Hemostatics
0
Factor VIII
9001-27-8
Types de publication
Case Reports
Journal Article
Review
Systematic Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
199-210Informations de copyright
© 2020 John Wiley & Sons Ltd.
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