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後天性血友病A：致死的な出血性疾患
Acquired Hemophilia A: A Potentially Fatal Bleeding Disorder.
PMID: 32714682 PMCID: PMC7377015. DOI: 10.7759/cureus.8744.
抄録
後天性血友病A（AHA）は、100万人あたり年間約1.5例の発症率を有する希少な自己免疫性血液疾患です。第VIII因子に対する自己抗体である第VIII因子インヒビターの発現により発症し、生命を脅かす可能性のある出血エピソードを引き起こします。AHAの診断はしばしば遅れ、困難である。我々は、最初に脳卒中の徴候や症状を呈した86歳の男性の症例を報告している。口腔粘膜出血と口腔底の腫脹を認めた。第VIII因子阻害剤のレベルが非常に高く（1152ベセスダ単位）、第VIII因子のレベルが非常に低い（1％未満）孤立性の活性化部分トロンボプラスチン時間（APTT）上昇を認めた。AHAは稀な自己免疫性出血性疾患であり、高齢者に多く見られる。AHAの出血は通常突然に起こり、時には生命を脅かすこともあるため、バイパス剤による早期止血と免疫抑制剤による治療を開始すべきである。この疾患は稀な疾患であるため、AHAの症例を報告することは、臨床医の意識を高め、早期診断・早期治療のための疑いの指標を高め、罹患率や死亡率の上昇を防ぐために非常に重要である。
Acquired hemophilia A (AHA) is a rare autoimmune hematological disorder that has an incidence of about 1.5 cases per million people per year. It occurs in the elderly with the median age of 75 years, and most of the cases are idiopathic. It occurs due to the development of factor VIII inhibitor, which is an autoantibody against factor VIII leading to potentially life-threatening bleeding episodes. The diagnosis of AHA is often delayed and challenging. We report a case of an 86-year-old male who initially presented with signs and symptoms of a stroke. He was found to have oral mucosal bleeding and swelling of the floor of the mouth. He later developed epistaxis, hematuria, and melena. He had an isolated elevation of activated partial thromboplastin time (APTT) with very high levels of factor VIII inhibitor (1152 Bethesda units) and very low levels of Factor VIII (<1%). He was managed with supportive transfusion, bypass agents, and immunosuppressive therapy. AHA is a rare autoimmune bleeding disorder and is more commonly seen in the elderly population. Bleeding in AHA is usually sudden and sometimes life-threatening. Hence early hemostasis with bypassing agents and treatment with immunosuppressive agents should be initiated. Due to the rarity of the disorder, it is crucial to report AHA cases to create awareness and increase the index of suspicion of the clinicians for early diagnosis and treatment to prevent morbidity and mortality.
Copyright © 2020, Singh et al.