期刊
JOURNAL OF PERSONALIZED MEDICINE
卷 12, 期 5, 页码 -出版社
MDPI
DOI: 10.3390/jpm12050678
关键词
hemophagocytic lymphohistiocytosis; ixazomib; multiple myeloma
资金
- Iuliu Hatieganu University School of Doctoral Studies
- Romanian Research Ministry [PCE 225/2021]
- Romanian National Ministry of Research, Innovation, and Digitalization [PN-III-P1-1.1-TE-2019-0271, PN-III-P1-1.1-PD-2019-0805, PD122/2020]
- PNCDI IV, Projects for Exploratory Medicine [PN-III-P4-ID-PCE-2020-1118]
- Projects for Exploratory Medicine [PCE 225/2021]
- PNCDI I-a collaboration between Romania and Belgium (Wallonia) [PN-III-CEI-BIM-PBE-2020-0016, 13-BM/2020]
- CCCDI-UEFISCDI within PNCDI III [PN-III-P4-ID-PCCF-2016-0112]
- international collaborative grant of the European Economic Space between Romania and Iceland 2020-2022: Cooperation strategy for knowledge transfer, internationalization and curricula innovation in the field of research education at the 3rd level of study
- project PDI-PFE-CDI 2021, entitled Increasing the Performance of Scientific Research, Supporting Excellence in Medical Research, and Innovation, PROGRES [40PFE/30.12.2021]
Hemophagocytic lymphohistiocytosis is a rare and life-threatening condition that requires thorough diagnosis and early treatment. Its development is associated with hematological malignancies, para-neoplastic syndromes, or infections related to immune system deficiency.
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 para-neoplastic 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.
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