Bullous pemphigoid is a common associated disorder with acquired haemophilia A
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ORIGINAL ARTICLE
Bullous pemphigoid is a common associated disorder with acquired haemophilia A Nurul Aidah Abdul‑Halim1 · Heng Joo Ng1 Received: 24 June 2020 / Revised: 31 August 2020 / Accepted: 2 September 2020 © Japanese Society of Hematology 2020
Abstract Although the estimated incidence of acquired haemophilia A (AHA) in Singapore is similar to those reported in the literature, we have observed differences in the frequency of their associated diseases, particularly bullous pemphigoid (BP). We investigated the actual incidence of BP among our AHA cohort, their clinical characteristics and treatment outcomes. 6 out of 37 (16%) patients with AHA had BP, making it the most common underlying disorder in our cohort. The median age at diagnosis of AHA was 76 years old with a female preponderance. Most patients had their AHA diagnosed after BP with the median time between BP to AHA diagnosis being 107.5 days. Initial haemostasis was achieved, and factor VIII inhibitor was eliminated in all patients with a median time of disappearance being 52 days. Two patients had recurrence of their factor VIII inhibitor during the tapering of their immunosuppression. There was no relationship between the relapse of BP and AHA. This study suggests that BP is a common association with AHA. These patients respond well to bypassing agent and immunosuppression. However, they have a higher recurrence of factor VIII inhibitor and should be monitored closely for relapse during the tapering period of their immunosuppression. Keywords Acquired haemophilia · Factor VIII inhibitor · Bleeding · Bypassing agents
Introduction Acquired haemophilia A (AHA) is a rare bleeding disorder, with an estimated incidence of 0.1–1.5 per million [1]. It is characterized by formation of antibodies or inhibitors against the endogenous coagulation factor VIII. Patients with this disorder may present with severe and potentially life-threatening haemorrhage. The most associated disorders are autoimmune disease, malignancy and pregnancy. However, in about 50% of the cases, no clear cause is identified. Bullous pemphigoid (BP) as an associated disease with AHA has been recognized, with 25 cases reported in the literature [2]. However, in large databases of AHA such as EACH2, UKHDCO and HTRS registries [3–5], such incidences are low or not reported, suggesting that its cooccurrence is rare. In HTRS registry of North America, for example, the incidence of BP amongst their 156 patients with AHA were only 2.1% [3]. * Nurul Aidah Abdul‑Halim [email protected] 1
Department of Haematology, Singapore General Hospital, 20 College Road, Singapore 169856, Singapore
Being a tertiary centre for both haematological and dermatological conditions, our hospital sees a fair number of patients with bleeding disorders and blistering skin diseases yearly. Our specialized coagulation laboratory also received samples from other hospitals in Singapore, and those with abnormal results will most often be referred to our department for further management. A
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