Constantinescu Catalin, Petrushev Bobe, Rus Ioana, Stefanescu Horia, Frasinariu Otilia, Margarit Simona, Dima Delia, Tomuleasa Ciprian
Department of Hematology, Iuliu Hatieganu University of Medicine and Pharmacy, 400349 Cluj-Napoca, Romania.
Department of Anesthesia and Intensive Care, Iuliu Hatieganu University of Medicine and Pharmacy, 400349 Cluj-Napoca, Romania.
J Pers Med. 2022 Apr 23;12(5):678. doi: 10.3390/jpm12050678.
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.
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、自然杀伤细胞（NK细胞）和先天性免疫系统的巨噬细胞发生病理性且不受控制的继发性激活。这种疾病可在与血液系统恶性肿瘤相关的散发性或家族性情况下发生，作为副肿瘤综合征，或与免疫系统缺陷相关的感染有关。这会导致引起整体临床表现的全身炎症。诊断应全面，且应尽快开始治疗。在当前的手稿中，我们专注于对HLH谱系进行分类，描述其病理生理学以及寻找和正确识别HLH所需的工具，以及当前的治疗机会。我们还介绍了首例在接受伊沙佐米-来那度胺-地塞米松方案治疗后发生HLH的多发性骨髓瘤患者。
