Hemophagocytic Lymphohistiocytosis in Adults
Hemophagocytic Lymphohistiocytosis in Adults
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DOI：
10.1016/j.hoc.2015.06.009
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发表时间：
2015-10-01
影响因子：
2.4
通讯作者：
Berliner, Nancy
中科院分区：
文献类型：
--
作者：
Campo, Meghan;Berliner, Nancy
噬血细胞性淋巴组织细胞增多症（HLH）是一种罕见但可能致命的病理性免疫失调综合征，其临床体征和症状为极端炎症。HLH可作为遗传性或散发性疾病发生，虽然被视为主要影响儿科人群的遗传性疾病，但可发生在任何年龄，并可与各种潜在疾病相关。临床表现为发热、肝脾肿大、细胞减少、造血器官巨噬细胞活化。治疗的重点是通过细胞毒性、免疫抑制治疗和治疗任何现有的HLH触发因素来抑制这种高炎症状态。
Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal syndrome of pathologic immune dysregulation characterized by clinical signs and symptoms of extreme inflammation. HLH can occur as a genetic or sporadic disorder and, though seen as an inherited condition affecting primarily a pediatric population, can occur at any age and can be encountered in association with a variety of underlying diseases. Clinically, the syndrome, whether genetic or acquired, is characterized by fever, hepatosplenomegaly, cytopenias, and activated macrophages in hematopoietic organs. Therapy centers on suppression of this hyperinflammatory state with cytotoxic, immunosuppressive therapy and treatment of any existing HLH triggers.