中文摘要
噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见、难以捉摸且危及生命的疾病,其特征是细胞毒性T细胞、NK 细胞(NK细胞)和固有免疫系统巨噬细胞的病理性且不受控制的继发性激活。该病可在散发性或家族性背景下发生,与血液系统恶性肿瘤相关,作为一种副肿瘤综合征,或与免疫系统缺陷相关的感染有关。这导致全身性炎症,从而引起整体临床表现。诊断应全面彻底,治疗应尽早启动。在本手稿中,我们聚焦于HLH谱系的分类,描述其病理生理学以及用于搜索和正确识别HLH所需的工具,以及当前的治疗机会。我们还呈现了首例多发性骨髓瘤患者在接受ixazomib-lenalidomide-dexamethasone方案治疗后发生HLH的病例。
展开英文摘要原文
Hemophagocytic lymphohistiocytosis (HLH) is a rare, elusive, and life-threatening condition that is characterized by the pathologic and uncontrolled secondary activation of the cytotoxic T-cells, natural killer cells (NK-cells), and macrophages of the innate immune system. This condition can develop in sporadic or familial contexts associated with hematological malignancies, as a paraneoplastic syndrome, or linked to an infection related to immune system deficiency.
This leads to the systemic inflammation responsible for the overall clinical manifestations. Diagnosis should be thorough, and treatment should be initiated as soon as possible. In the current manuscript, we focus on classifying the HLH spectrum, describing the pathophysiology and the tools needed to search for and correctly identify HLH, and the current therapeutic opportunities.
We also present the first case of a multiple myeloma patient that developed HLH following therapy with the ixazomib-lenalidomide-dexamethasone protocol.
论文信息
- 作者
- Constantinescu C、Petrushev B、Rus I、Stefanescu H、Frasinariu O、Margarit S、Dima D、Tomuleasa C
- 单位
- Department of Hematology, Iuliu Hatieganu University of Medicine and Pharmacy, 400349 Cluj-Napoca, Romania.Italy
- 文献类型
- 综述
- 期刊
- Journal of personalized medicine2022 Apr 23