联合免疫抑制治疗合并潜在结核的获得性血友病A的快速和令人满意的结果。

IF 0.7 Q4 HEMATOLOGY Case Reports in Hematology Pub Date : 2022-01-01 DOI:10.1155/2022/2271228
G A C Gamakaranage, C Kulathilake, P G N Nadeeshani, K H B P Fernandopulle, C S Moonesinghe, D Gunawardena, R S Gamage, N Perera, J Indrakumar
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引用次数: 0

摘要

获得性血友病A (AHA)是一种罕见的疾病,在英国每年的发病率为每百万人1.5例。由于诊断困难以及低滴度抑制剂水平的患者无症状,发生率可能被低估。它通常是一种影响老年人的疾病,但已知在年轻人群中出现疾病高峰。常见的潜在疾病是自身免疫性疾病、恶性肿瘤、感染和药物。然而,约50%的病例没有特定的病因,约10%的病例不会有出血表现。因此,应评估单独的APTT延长,特别是在任何止血挑战之前。我们报告一例中年男子谁提出出血由于AHA相关的高抑制滴度和活动性肺结核。他接受了抗结核和联合积极免疫抑制治疗,结果令人满意的疾病缓解。
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A Rapid and Satisfactory Outcome with Combined Immunosuppressive Therapy in Acquired Haemophilia A with Underlying Tuberculosis.

Acquired haemophilia A (AHA) is a rare disorder with an incidence of 1.5 cases per million per year in the United Kingdom. The incidence could be underestimated due to difficulty in diagnosis and also due to the fact that people with low titre inhibitor levels are asymptomatic. It is usually a disease affecting elderly but a disease peak in the younger population is known. The common underlying diseases are autoimmune disorders, malignancies, infections, and drugs. However, approximately 50% of the cases do not have a specific aetiology and about 10% will not have bleeding manifestations. Therefore, an isolated prolongation of APTT should be evaluated, especially prior to any haemostatic challenges. We report a case of a middle-aged man who presented with bleeding due to AHA associated with high inhibitory titres and active pulmonary tuberculosis. He was treated with both antituberculous and combined-aggressive immunosuppressive therapy which resulted in satisfactory disease remission.

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审稿时长
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