获得性血友病 A (AHA):孟加拉国达卡一家三级医院中的罕见出血性疾病

Md Abdul Quader, Anowar Ahmed Miajee, Nripen Kumar Kundu, Md Shamsuzzaman Shaheen
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摘要

获得性血友病 A(AHA)是一种罕见的出血性疾病。这是由于因子 VIII (FVIII) 的自身抗体中和了因子 VIII 的能力。这可能导致严重的出血发作,具有很高的发病率和死亡率。获得性血友病通常发生在 60 岁以上或产后,并伴有其他自身免疫性疾病。如果患者新近出现出血症状,且之前没有个人或家族出血史,则应怀疑诊断为后天性血友病。这种疾病的特征是粘膜出血,导致瘀斑、恶露、血肿、血尿和伤口自发渗出。治疗时应同时采用止血疗法和免疫调节策略,前者可最大限度地减少出血,后者则可重建对第八因子的免疫耐受。在此,我们介绍一例年龄较大的男性获得性血友病 A 患者,既往无抗凝剂治疗史,也无出血发作的家族史或个人史。患者因股骨颈骨折接受了半关节置换术,植入了骨水泥假体。术后第 10 天,他被诊断为原位导管内血块滞留,术后第 12 天伤口出现渗血。他的化验报告显示凝血酶原时间(PT)和活化部分凝血酶原时间(APTT)延长。他的第八因子水平很低,第八因子抑制剂显示存在时间依赖性抑制剂。因此,他被诊断为获得性血友病 A(AHA),这在孟加拉国非常罕见。通过药物治疗,他的第八因子水平恢复正常。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Acquired Hemophilia A (AHA): A Rare Bleeding Disorder In a Tertiary Care Hospital In Dhaka, Bangladesh
Acquired Hemophilia A (AHA) is a rare bleeding disorder. This results from autoantibodies to factor VIII (FVIII) that neutralizes the ability of factor VIII. This causes potentially severe bleeding episodes that carries a high risk of morbidity and mortality. Acquired Hemophilia usually occurs over 60 years of age or in postpartum period and associated with other autoimmune conditions. The diagnosis should be suspected when patients with new onset of bleeding without prior personal or family history of bleeding. The hallmark of this condition is mucocutaneous bleeding leading to ecchymosis, malena, hematoma, hematuria and spontaneous oozing from wounds. Management should uphold both hemostatic therapies to minimize bleeding and immune modulation strategies to re-establish immune tolerance to factor VIII. Here we present an older male case of such Acquired Hemophilia A without history of previous anticoagulant therapy as well as family history or personal history of bleeding episodes. The patient has got hemiarthroplasty with prosthesis with bone cementing due to fracture neck of femur. After 10th post-operative day he was diagnosed with clot retention in catheter in-situ and oozing from wound site after 12th post-operative day. His laboratory reports showed prolongation of Prothrombin Time (PT) and Activated Partial Prothrombin Time (APTT). His factor VIII was very low level and factor VIII inhibitor showed the time depended inhibitor presence. Thus diagnosed as Acquired Hemophilia A (AHA), the rare case in Bangladesh. He was managed with medications that resulted in normalization of factor VIII level. J MEDICINE 2024; 25: 173-175
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