open access

Vol 13, No 3 (2020)
Review paper
Published online: 2020-12-03
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A practical approach to the patient with acquired haemophilia A in Poland

Magdalena Górska-Kosicka, Michał Wójciak, Jerzy Windyga
DOI: 10.5603/JTM.2020.0007
·
Journal of Transfusion Medicine 2020;13(3):186-194.

open access

Vol 13, No 3 (2020)
REVIEW ARTICLE
Published online: 2020-12-03

Abstract

Acquired haemophilia (AHA) is a severe acquired bleeding disorder that develops due to circulating autoantibodies directed against coagulation factor VIII (FVIII). These antibodies inhibit the coagulation activity of FVIII in patient’s plasma. AHA is mainly diagnosed in the elderly and more than half of the cases are idiopathic. Underlying conditions which favor AHA occurrence are — among others — autoimmune diseases, neoplasms, pregnancy and 12-month post-partum period. The clinical picture is dominated by extensive ecchymosis, less often by intramuscular and retroperitoneal haematomas or hemorrhages to the central nervous system. Laboratory findings include isolated prolonged activated partial thromboplastin time (APTT), decreased FVIII activity and the presence of a FVIII inhibitor. Although patients with AHA usually present with severe hemorrhagic disorders, asymptomatic forms may also occur, with accidentally detected prolonged APTT as the only abnormality. The aim of AHA management is bleeding control and eradication of inhibitor.

Abstract

Acquired haemophilia (AHA) is a severe acquired bleeding disorder that develops due to circulating autoantibodies directed against coagulation factor VIII (FVIII). These antibodies inhibit the coagulation activity of FVIII in patient’s plasma. AHA is mainly diagnosed in the elderly and more than half of the cases are idiopathic. Underlying conditions which favor AHA occurrence are — among others — autoimmune diseases, neoplasms, pregnancy and 12-month post-partum period. The clinical picture is dominated by extensive ecchymosis, less often by intramuscular and retroperitoneal haematomas or hemorrhages to the central nervous system. Laboratory findings include isolated prolonged activated partial thromboplastin time (APTT), decreased FVIII activity and the presence of a FVIII inhibitor. Although patients with AHA usually present with severe hemorrhagic disorders, asymptomatic forms may also occur, with accidentally detected prolonged APTT as the only abnormality. The aim of AHA management is bleeding control and eradication of inhibitor.

Get Citation

Keywords

acquired haemophilia A; factor VIII; bypassing agents; inhibitor; bleeding

About this article
Title

A practical approach to the patient with acquired haemophilia A in Poland

Journal

Journal of Transfusion Medicine

Issue

Vol 13, No 3 (2020)

Article type

Review paper

Pages

186-194

Published online

2020-12-03

DOI

10.5603/JTM.2020.0007

Bibliographic record

Journal of Transfusion Medicine 2020;13(3):186-194.

Keywords

acquired haemophilia A
factor VIII
bypassing agents
inhibitor
bleeding

Authors

Magdalena Górska-Kosicka
Michał Wójciak
Jerzy Windyga

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