大疱性类天疱疮 (BP) 是一种罕见的水疱性疾病，通常被认为是副肿瘤综合征的主要症状。回顾性研究已确定其与血液恶性肿瘤，特别是淋巴增殖性疾病的联系。在这里，我们介绍了一位 28 岁男性患者成功同时治疗 BP 和新发急性髓性白血病 (AML) 的首例。鉴于这两种情况的罕见性和严重性，我们的治疗策略旨在通过将免疫抑制治疗（首先使用高剂量皮质类固醇进行血浆置换，然后使用抗 CD20 单克隆抗体和静脉注射免疫球蛋白 2 g/m2）与利用氟达拉滨的淋巴细胞清除抗白血病化疗相结合来最大限度地提高疗效（FLAG-IDA 诱导方案）。诊断后，考虑到患者年轻且同时存在两种罕见且可能危及生命的疾病，我们选择了积极治疗。在实现 AML 的完全形态学缓解且可测量残留病 (MRD) 阴性后，尽管血压未完全消退，我们继续进行高剂量阿糖胞苷巩固治疗，然后进行外周干细胞收获和自体干细胞移植 (ASCT)。我们的 ASCT 预处理方案涉及 Bu-Cy 并添加抗胸腺细胞球蛋白。 ASCT 后第 100 天，骨髓评估证实形态学缓解和 MRD 阴性。与此同时，随着 BP180 抗体水平的正常化，BP 已完全解决。© 2024。作者获得 Springer-Verlag GmbH 德国（Springer Nature 旗下公司）的独家许可。

Bullous pemphigoid (BP) is a rare blistering disease often considered a primary sign of a paraneoplastic syndrome. Retrospective studies have established its link with hematological malignancies, particularly lymphoproliferative disorders. Here, we present what we believe to be the inaugural case of successful simultaneous management of BP and de novo acute myeloid leukemia (AML) in a 28-year-old male patient. Given the rarity and severity of both conditions, our treatment strategy aimed to maximize efficacy by combining immunosuppressive therapy (initially plasmapheresis with high-dose corticosteroids, followed by anti-CD20 monoclonal antibody and intravenous immunoglobulins 2 g/m2) with lymphodepleting antileukemic chemotherapy utilizing Fludarabine (FLAG-IDA induction regimen). Following diagnosis, considering the patient's youth and the concurrent presence of two rare and potentially life-threatening diseases, we opted for an aggressive treatment. Upon achieving complete morphological remission of AML with measurable residual disease (MRD) negativity, despite incomplete resolution of BP, we proceeded with high-dose cytarabine consolidation followed by peripheral stem cell harvest and autologous stem cell transplantation (ASCT). Our conditioning regimen for ASCT involved Bu-Cy with the addition of anti-thymocyte globulins. At day + 100 post-ASCT, bone marrow evaluation confirmed morphological remission and MRD negativity. Meanwhile, BP had completely resolved with normalization of BP180 antibody levels.© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.