Epidemiology, spectrum of clinical manifestations and diagnostic issue of acquired haemophilia: A case series
- Author:
Wan Suriana Wan Ab Rahman
1
Author Information
1. School of Dental Sciences, Universiti Sains Malaysia, 16150 Kubang Kerian Kelantan
- Collective Name:Wan Suriana Wan Ab Rahman; Wan Zaidah Abdullah; Azlan Husin; Nik Fatma Fairuz Nik Mohd Hassan; Mohd Nazri Hassan; Zefarina Zulkafli
- Publication Type:Case Reports
- Keywords:
Acquired haemophilia A;
factor VIII inhibitor;
haemophilia;
bleeding
- MeSH:
haemophilia
- From:The Malaysian Journal of Pathology
2019;41(2):185-189
- CountryMalaysia
- Language:English
-
Abstract:
Introduction: Acquired haemophilia A (AHA) is a rare acquired bleeding disorder caused by polyclonal immunoglobulin G autoantibodies against clotting factor VIII (FVIII). The incidence was reported to be rare occurring in 0.2- 4 cases/million/year. Patients may present with different clinical manifestations to various specialties. Early recognition of the disease contributes to favourable clinical outcome. Case Series: Here, we reported five cases of this disorder with different clinical presentations from two tertiary hospitals in Kelantan state, Malaysia within a two year-period. Most of them were elderly, except for one who presented at the age of 36 years old. No direct or secondary cause was identified except for one patient who had developed from pregnancy-related at 3 weeks postpartum. These patients presented with spontaneous bleeding typically into skin, muscles, and mucous membranes but also at rare site in the epidural space. All patients denied previous history of bleeding or family history of bleeding disorder. FVIII activities were recorded between <1% to 19%, while the inhibitor titre levels were between 3.9 BU to 340 BU. The treatment approaches especially at presentation were complicated by unfamiliarity of managing this rare condition but all these patients received appropriate medical attention. Discussion: Prompt diagnosis and management in the right hand are critical. Awareness of this disorder by medical personnel at all levels in the community and in various specialties is important.
- Full text:5.2019my1009.pdf
