Hemophagocytic lymphohistiocytosis as an etiology of bone marrow failure

被引：7
作者：
Paolino, Jonathan [1 ]
Berliner, Nancy [2 ]
Degar, Barbara [1 ]
机构：
[1] Harvard Med Sch, Dana Farber Canc Inst, Dept Pediat Oncol, Boston, MA 02115 USA
[2] Harvard Med Sch, Brigham & Womens Hosp, Div Hematol, Boston, MA USA
来源：
FRONTIERS IN ONCOLOGY | 2022年 / 12卷
关键词：
hemophagocytic lymphohistiocytosis; hypercytokinaemia; familial hemophagocytic lymphohistiocytosis (FHL); macrophage activation syndrome (MAS); cytopenia; bone marrow failure (BMF); MACROPHAGE ACTIVATION SYNDROME; JUVENILE IDIOPATHIC ARTHRITIS; INTERFERON-GAMMA; IFN-GAMMA; CYTOKINE PATTERN; XIAP DEFICIENCY; T-CELLS; MUTATIONS; DIAGNOSIS; CHILDREN;
D O I：
10.3389/fonc.2022.1016318
中图分类号：
R73 [肿瘤学];
学科分类号：
100214 ;
摘要：
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of multiorgan system dysfunction that is caused by hypercytokinemia and persistent activation of cytotoxic T lymphocytes and macrophages. A nearly ubiquitous finding and a diagnostic criterion of HLH is the presence of cytopenias in >= 2 cell lines. The mechanism of cytopenias in HLH is multifactorial but appears to be predominantly driven by suppression of hematopoiesis by pro-inflammatory cytokines and, to some extent, by consumptive hemophagocytosis. Recognition of cytopenias as a manifestation of HLH is an important consideration for patients with bone marrow failure of unclear etiology.
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页数：9
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