Long-Term Experience with Acquired Haemophilia A - A 40-Year Single-Centre Study of Clinical Features and Outcome

Sep 30, 2026

Acquired haemophilia A (AHA) is a rare autoimmune disorder in which autoantibodies against factor VIII activity reduce its functionality, often causing unexpected aPTT prolongation and spontaneous bleeding without personal or family bleeding history. A single-centre cohort of 35 patients (1984–2024) examined demographics, underlying conditions, bleeding patterns, therapies, and outcomes. Most patients had treatment with corticosteroids alone or combined with cyclophosphamide. FVIII inhibitor titre > 20 BU emerged as the only significant predictor for time to complete remission, supporting timely referral and experienced laboratory-supported care.

LanguageEnglish

CategoryResearch & Report

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Pages13

Reading Time33 min

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Abstract

1. Introduction

Article Long-Term Experience with Acquired Haemophilia A: A 40-Year Single-Centre Study of Clinical Features and Outcome Daniele Roselli 1, Giuseppe Malcangi 2, Maria Addolorata Bonifacio 3, Prudenza Ranieri 1, Renato Marino 2 and Maria Addolorata Mariggiò 1,3, * Academic Editor: Moshe Mittelman Received: 17 November 2025 Revised: 20 December 2025 Accepted: 24 December 2025 Published: 26 December 2025 Copyright: © 2025 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license. 1 Division of University Clinical Pathology, University Hospital Policlinico Consorziale, 70124 Bari, Italy; [rosellidaniele94@gmail.com](rosellidaniele94@gmail.com) (D.R.) 2 Haemophilia and Thrombosis Centre, University Hospital Policlinico Consorziale, 70124 Bari, Italy 3 Section of Clinical Pathology, Department of Precision and Regenerative Medicine and Ionian Area, University of Bari Aldo Moro Medical School, 70124 Bari, Italy * [Correspondence: mariaaddolorata.mariggio@uniba.it](Correspondence: mariaaddolorata.mariggio@uniba.it) Abstract Background: Acquired haemophilia A (AHA) is a rare autoimmune disorder characterized by the development of autoantibodies against Factor VIII activity, leading to a significant reduction in its functionality. Clinically, AHA presents with an unexpected prolongation of activated partial thromboplastin time (aPTT) and spontaneous bleeding episodes in patients without any personal or family history of haemorrhages. Bleeding manifestations can be severe at presentation, making early diagnosis and prompt treatment essential to reduce morbidity and mortality. Methods: We report on a single-centre cohort of 35 patients with AHA (examined from 1984 to 2024), analysing their demographics, underlying conditions, bleeding characteristics, treatment and outcome. Results: The median age of patients at diagnosis was 69 years (ranging from 18 to 92), 15 were males and 20 females. AHA wasidiopathic in 37% of cases, severe bleeding was observed in 54% of patients treated with bypassing agents. Recombinant activated Factor VII (rFVIIa) was administered in 79% of cases and activated prothrombin complex concentrate (aPCC) in 10%, with no significant differences in haemostatic response and no thromboembolic complications. Occurrence of major bleeding showed no significant association with sex, age group, underlying condition, baseline Factor VIII activity or inhibitor titre at diagnosis. A total of 69% of patients were treated with corticosteroids alone, and 23% received a combination of corticosteroids and cyclophosphamide. Two patients died, six were lost to follow-up after partial remission, and one relapsed without bleeds after complete remission. Statistical analyses highlighted that the FVIII inhibitor titre > 20 BU was the only significant prognostic factor affecting time to complete remission. Conclusions: These observations emphasize the critical role of clinical suspicion and timely referral to experienced centres with adequate laboratory support for the effective management of AHA. Keywords: haemophilia A; factor VIII; inhibitor; prognosis; treatment; haemorrhage 1. Introduction Acquired Haemophilia A (AHA) is a rare autoimmune disease with an incidence of approximately 1.5 cases per million persons per year [1] . It is caused by the development of inhibitory autoantibodies against the plasmatic factor VIII activity (FVIII:C) leading to the typical haemorrhagic symptoms in patients without a family or personal history of bleeding [2] . The incidence of AHA typically exhibits a biphasic distribution, with one peak associated with pregnancy and a second occurring in individuals over 60 years of age. Approximately 50% of patients with AHA present with underlying conditions, most commonly other autoimmune diseases (rheumatoid arthritis, systemic lupus erythematosus and

FAQs

What defines acquired haemophilia A in the study?

AHA is characterized by inhibitory autoantibodies against factor VIII activity, leading to reduced FVIII functionality, unexpected aPTT prolongation, and spontaneous bleeding in patients without a bleeding history.

How many patients and what time span were analyzed?

The study analyzed a single-centre cohort of 35 AHA patients examined from 1984 to 2024.

Which factor significantly affected time to complete remission?

An FVIII inhibitor titre greater than 20 BU was the only significant prognostic factor influencing time to complete remission.

Sep 30, 2026

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