Pregnancy-associated acquired haemophilia A
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Pregnancy-associated acquired haemophilia A : results from the European Acquired Haemophilia (EACH2) registry. / Tengborn, L; Baudo, F; Huth-Kühne, A; Knoebl, P; Lévesque, H; Marco, P; Pellegrini, F; Nemes, L; Collins, P; EACH2 registry contributors ; Holstein, Katharina.
In: BJOG-INT J OBSTET GY, Vol. 119, No. 12, 01.11.2012, p. 1529-37.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Pregnancy-associated acquired haemophilia A
T2 - results from the European Acquired Haemophilia (EACH2) registry
AU - Tengborn, L
AU - Baudo, F
AU - Huth-Kühne, A
AU - Knoebl, P
AU - Lévesque, H
AU - Marco, P
AU - Pellegrini, F
AU - Nemes, L
AU - Collins, P
AU - EACH2 registry contributors
AU - Holstein, Katharina
N1 - © 2012 The Authors BJOG An International Journal of Obstetrics and Gynaecology © 2012 RCOG.
PY - 2012/11/1
Y1 - 2012/11/1
N2 - OBJECTIVE: The European Acquired Haemophilia registry (EACH2) collected data on the demographics, diagnosis, underlying disorders, bleeding characteristics, treatment, and outcome of women with acquired haemophilia A (AHA), a rare and often severe bleeding disorder caused by autoantibodies directed against coagulation factor VIII.DESIGN: Prospective, multi-centre, large-scale, pan-European registry.SETTING: A total of 117 haemophilia centres in 13 European countries.POPULATION: Pregnancy-associated AHA.METHODS: Data were reported using a web-based electronic case report form. Diagnosis was based on the presence of a prolonged activated partial thromboplastin time, reduced coagulation Factor VIII level and positive inhibitor assay.MAIN OUTCOME MEASURES: Presenting characteristics, time to diagnosis, haemostatic treatment and outcome, immunosuppressive treatment and outcome.RESULTS: The EACH2 registry (n = 501) documented 42 (8.4%) cases of AHA associated with the peripartum period, a median Factor VIII level at diagnosis of 2.5 (range 0-25) IU/dl and inhibitor titre of 7.8 (range 0.7-348) BU/ml. Antepartum inhibitors were evident in eight women. Time to diagnosis of AHA after delivery was 89 (range 21-120) days. First-line haemostatic treatment was successful in 20/23 (87%) women treated. Bleeding episodes resolved in 17/18 (94%) women treated with a bypassing agent and 29/39 (74%) women achieved complete remission with first-line immunosuppressive treatment. Two babies experienced postnatal bleeding, suggesting transplacental transfer of the antibody. All women were alive at last follow-up.CONCLUSIONS: Although rare, pregnancy-associated AHA may cause severe bleeding-related morbidity. Once diagnosed, women respond well to haemostatic treatment with bypassing agents and immunosuppression. Awareness of peripartum AHA requires improvement to facilitate rapid and appropriate management.
AB - OBJECTIVE: The European Acquired Haemophilia registry (EACH2) collected data on the demographics, diagnosis, underlying disorders, bleeding characteristics, treatment, and outcome of women with acquired haemophilia A (AHA), a rare and often severe bleeding disorder caused by autoantibodies directed against coagulation factor VIII.DESIGN: Prospective, multi-centre, large-scale, pan-European registry.SETTING: A total of 117 haemophilia centres in 13 European countries.POPULATION: Pregnancy-associated AHA.METHODS: Data were reported using a web-based electronic case report form. Diagnosis was based on the presence of a prolonged activated partial thromboplastin time, reduced coagulation Factor VIII level and positive inhibitor assay.MAIN OUTCOME MEASURES: Presenting characteristics, time to diagnosis, haemostatic treatment and outcome, immunosuppressive treatment and outcome.RESULTS: The EACH2 registry (n = 501) documented 42 (8.4%) cases of AHA associated with the peripartum period, a median Factor VIII level at diagnosis of 2.5 (range 0-25) IU/dl and inhibitor titre of 7.8 (range 0.7-348) BU/ml. Antepartum inhibitors were evident in eight women. Time to diagnosis of AHA after delivery was 89 (range 21-120) days. First-line haemostatic treatment was successful in 20/23 (87%) women treated. Bleeding episodes resolved in 17/18 (94%) women treated with a bypassing agent and 29/39 (74%) women achieved complete remission with first-line immunosuppressive treatment. Two babies experienced postnatal bleeding, suggesting transplacental transfer of the antibody. All women were alive at last follow-up.CONCLUSIONS: Although rare, pregnancy-associated AHA may cause severe bleeding-related morbidity. Once diagnosed, women respond well to haemostatic treatment with bypassing agents and immunosuppression. Awareness of peripartum AHA requires improvement to facilitate rapid and appropriate management.
KW - Adult
KW - Antifibrinolytic Agents
KW - Blood Coagulation Factors
KW - Drug Therapy, Combination
KW - Europe
KW - Factor VIIa
KW - Female
KW - Follow-Up Studies
KW - Hemophilia A
KW - Hemostatics
KW - Humans
KW - Immunosuppressive Agents
KW - Kaplan-Meier Estimate
KW - Pregnancy
KW - Pregnancy Complications, Hematologic
KW - Prospective Studies
KW - Recombinant Proteins
KW - Registries
KW - Treatment Outcome
U2 - 10.1111/j.1471-0528.2012.03469.x
DO - 10.1111/j.1471-0528.2012.03469.x
M3 - SCORING: Journal article
C2 - 22901076
VL - 119
SP - 1529
EP - 1537
JO - BJOG-INT J OBSTET GY
JF - BJOG-INT J OBSTET GY
SN - 1470-0328
IS - 12
ER -