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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, Jahrgang 119, Nr. 12, 01.11.2012, S. 1529-37.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Tengborn, L, Baudo, F, Huth-Kühne, A, Knoebl, P, Lévesque, H, Marco, P, Pellegrini, F, Nemes, L, Collins, P, EACH2 registry contributors & Holstein, K 2012, 'Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry', BJOG-INT J OBSTET GY, Jg. 119, Nr. 12, S. 1529-37. https://doi.org/10.1111/j.1471-0528.2012.03469.x

APA

Tengborn, L., Baudo, F., Huth-Kühne, A., Knoebl, P., Lévesque, H., Marco, P., Pellegrini, F., Nemes, L., Collins, P., EACH2 registry contributors, & Holstein, K. (2012). Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry. BJOG-INT J OBSTET GY, 119(12), 1529-37. https://doi.org/10.1111/j.1471-0528.2012.03469.x

Vancouver

Tengborn L, Baudo F, Huth-Kühne A, Knoebl P, Lévesque H, Marco P et al. Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry. BJOG-INT J OBSTET GY. 2012 Nov 1;119(12):1529-37. https://doi.org/10.1111/j.1471-0528.2012.03469.x

Bibtex

@article{1b726e2aee5e4c0eba17660ae5190c68,
title = "Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry",
abstract = "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.",
keywords = "Adult, Antifibrinolytic Agents, Blood Coagulation Factors, Drug Therapy, Combination, Europe, Factor VIIa, Female, Follow-Up Studies, Hemophilia A, Hemostatics, Humans, Immunosuppressive Agents, Kaplan-Meier Estimate, Pregnancy, Pregnancy Complications, Hematologic, Prospective Studies, Recombinant Proteins, Registries, Treatment Outcome",
author = "L Tengborn and F Baudo and A Huth-K{\"u}hne and P Knoebl and H L{\'e}vesque and P Marco and F Pellegrini and L Nemes and P Collins and {EACH2 registry contributors} and Katharina Holstein",
note = "{\textcopyright} 2012 The Authors BJOG An International Journal of Obstetrics and Gynaecology {\textcopyright} 2012 RCOG.",
year = "2012",
month = nov,
day = "1",
doi = "10.1111/j.1471-0528.2012.03469.x",
language = "English",
volume = "119",
pages = "1529--37",
journal = "BJOG-INT J OBSTET GY",
issn = "1470-0328",
publisher = "Wiley-Blackwell",
number = "12",

}

RIS

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 -