Department of Hematology, Iuliu Hatieganu University of Medicine and Pharmacy Cluj Napoca, Romania; Department of Anesthesia and Intensive Care, Clinical Emergency County Hospital, Cluj Napoca, Romania; Department of Anesthesia and Intensive Care, Iuliu Hatieganu University of Medicine and Pharmacy Cluj Napoca, Romania; Medfuture Research Center for Advanced Medicine, Iuliu Hatieganu University of Medicine and Pharmacy Cluj Napoca, Romania.
Department of Hematology, Iuliu Hatieganu University of Medicine and Pharmacy Cluj Napoca, Romania; Medfuture Research Center for Advanced Medicine, Iuliu Hatieganu University of Medicine and Pharmacy Cluj Napoca, Romania; Department of Hematology, Ion Chiricuta Clinical Cancer Center, Cluj Napoca, Romania.
Blood Rev. 2022 May;53:100907. doi: 10.1016/j.blre.2021.100907. Epub 2021 Nov 10.
There is a dire need to develop an algorithm to improve the recognition of acquired hemophilia A and B (AHA and AHB) in clinical practice. Initial and intensive care unit (ICU) management of the disorder is particular and represents a challenge for the internist/hematologist and the ICU physician. A delay in the proper treatment of bleeding episodes can lead to a life-threatening event. Expert advice should be sought as soon as possible. Succesful resolution involves accurate diagnosis, bleeding control with hemostatic and immunotherapy, and eradication of the autoantibodies to improve overall survival. Current treatment guidelines are based on the literature in the form of cases and observational studies due to a lack of randomized controlled trials. AH can be triggered by many pathologies, presenting as a paraneoplastic syndrome in case of malignancies or as surgical associated acquired hemophilia (SAHA). We have reviewed the literature from 2015 to 2021 regarding the new case reports to further assess if there is an improvement in the clinical approach.
目前迫切需要开发一种算法,以提高在临床实践中识别获得性血友病 A 和 B(AHA 和 AHB)的能力。该病的初始治疗和重症监护病房(ICU)管理具有特殊性,这对内科医生/血液科医生和 ICU 医生来说是一个挑战。如果对出血发作的治疗不及时,可能会导致危及生命的事件。应尽快寻求专家建议。成功解决该问题需要准确的诊断、通过止血和免疫疗法控制出血,并消除自身抗体,从而提高整体生存率。由于缺乏随机对照试验,目前的治疗指南是基于病例和观察性研究的文献制定的。AH 可由多种疾病引发,在恶性肿瘤的情况下表现为副肿瘤综合征,或作为手术相关获得性血友病(SAHA)。我们回顾了 2015 年至 2021 年的新病例报告文献,以进一步评估临床方法是否有所改善。