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

Dewarrat, Natacha; Gavillet, Mathilde; Angelillo-Scherrer, Anne; Naveiras, Olaia; Grandoni, Francesco; Tsakiris, Dimitrios A; Alberio, Lorenzo; Blum, Sabine (2021). Acquired haemophilia A in the postpartum and risk of relapse in subsequent pregnancies: A systematic literature review. Haemophilia, 27(2), pp. 199-210. Wiley 10.1111/hae.14233

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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.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Haematology and Central Haematological Laboratory
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > Unit Childrens Hospital > Forschungsgruppe Hämatologie (Erwachsene)

UniBE Contributor:

Angelillo, Anne

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1365-2516

Publisher:

Wiley

Language:

English

Submitter:

Pierrette Durand Lüthi

Date Deposited:

18 Feb 2021 08:13

Last Modified:

22 Mar 2021 01:34

Publisher DOI:

10.1111/hae.14233

PubMed ID:

33550699

Uncontrolled Keywords:

Factor VIII deficiency Haemophilia A acquired postpartum haemorrhage postpartum period recurrence

BORIS DOI:

10.48350/152393

URI:

https://boris.unibe.ch/id/eprint/152393

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