Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder caused by antibodies which neutralize the function of factor VIII (FVIII). The disease presents a complex clinical challenge to the treating Physicians and Hematologists. As the disease is associated with high mortality, prompt management is necessary. Early recognition, quick diagnosis and timely referral to a specialized center are important for better management of these patients. The different clinical manifestations, underlying pathology, inhibitor kinetics and the associated age related comorbidities do not allow extrapolation of the treatment protocols of congenital hemophilia to AHA. The basic strategies of the management of AHA patients involve maintaining hemostasis, suppression or eradication of antibodies, diagnosis and treatment of underlying pathology and avoid treatment related complications like thrombosis. The efficiency of hemostatic agents which are generally used to treat AHA is unpredictable. Due to the rarity of the disease, there are no randomized clinical trials on the management of this disorder and thus the expertise and experience of the treating Physicians' guide treatment strategies.
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Laboratory Assessment of Factor VIII Inhibitors: When Is It Required? A Perspective Informed by Local Practice.
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Haematology, Sydney Centres for Thrombosis and Haemostasis, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead, NSW 2145, Australia.
This perspective discusses the critical role of laboratory assessments in assessing factor VIII (FVIII) inhibitors. These are auto- and alloantibodies that can develop against both endogenous and exogenous FVIII, respectively. Assessment for inhibitors represents a key part of the management of both congenital hemophilia A (CHA), an inherited deficiency, and acquired hemophilia A (AHA), an autoimmune condition.
View Article and Find Full Text PDF[Clinical Analysis of 25 Cases of Acquired Hemophilia A in a Single Center].
Zhongguo Shi Yan Xue Ye Xue Za Zhi
December 2024
Department of Hematology, The Second Hospital of Hebei Medical University, Hebei Key Laboratory of Hematology, Shijiazhuang 050000, Hebei Province, China.
Objective: To explore the diagnosis and treatment of acquired hemophilia A (AHA) based on the analysis of clinical data.
Methods: A retrospective analysis was conducted on the clinical manifestations, laboratory characteristics, treatment, and outcomes of 25 patients diagnosed with AHA who were admitted to the Second Hospital of Hebei Medical University.
Results: Among all patients, 11 cases had secondary factors, including 5 cases of autoimmune diseases, 3 cases of pregnancy-related disease, 1 case of pemphigoid, 1 case of Graves' disease, and 1 case of monoclonal gammaglobulinemia of unknown significance (MGUS).
Real-World Data on Patients With Acquired Haemophilia A in Japan Undergoing Rehabilitation or With Low Activities of Daily Living Scores: The ORIHIME II Study.
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Department of Pediatrics, Nara Medical University, Kashihara, Japan.
Introduction: Acquired haemophilia A (AHA) is characterized by the development of autoantibodies against factor VIII, reducing its activity and potentially resulting in bleeding.
Aim: To assess the characteristics of people with AHA undergoing rehabilitation and/or with low activities of daily living (ADL) scores, thereby characterizing unmet needs in the management of AHA and informing treatment optimization.
Methods: ORIHIME II, the largest epidemiological and treatment survey of AHA in Japan, is a descriptive, retrospective, observational study conducted using health claims data from April 2008 to October 2021.
Pediatric IgG4-related dacryoadenitis and sialadenitis (Mikulicz's disease) with acquired hemophilia A: A case report and review of literature.
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Pediatrics Department and Department of Pathology, Hanoi Medical University, Hanoi, Vietnam.
We report a case of IgG4-related dacryoadenitis and sialadenitis (Mikulicz's disease) with acquired hemophilia A (AHA) in a pediatric patient. An 11-year-old female presented with intermittent swelling of the bilateral upper eyelids and neck areas. Clinical examination revealed bilateral swollen upper eyelids with palpable mass lesions in the lacrimal fossa.
View Article and Find Full Text PDFOutcomes of Emicizumab in Acquired Hemophilia Patients: A Systematic Review.
Clin Appl Thromb Hemost
November 2024
Faculty of Medecine of Monastir Tunisia, University of Monastir, Monastir, Tunisia.
Background: Emicizumab, a bispecific factor VIII mimetic antibody, was approved in 2018 for bleeding prophylaxis in congenital hemophilia A with or without inhibitors. Since then, several case reports and case series have described the off-label use of emicizumab in acquired hemophilia A (AHA), and data from two clinical trials were recently published (AGEHA, GTH-AHA-EMI).
Objectives: To describe the reported data on the outcomes of emicizumab, highlighting its benefit/risk profile in treatment.
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