Infection- and malignancy-associated hemophagocytic syndromes - Secondary hemophagocytic lymphohistiocytosis
Infection- and malignancy-associated hemophagocytic syndromes - Secondary hemophagocytic lymphohistiocytosis
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DOI：
10.1016/s0889-8588(05)70521-9
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发表时间：
1998-04-01
影响因子：
2.4
通讯作者：
Henter, JI
中科院分区：
文献类型：
--
作者：
Janka, G;Imashuku, S;Henter, JI
噬血细胞淋巴组织细胞增生症是一系列不同的病理性疾病，在这些疾病中，T细胞引起吞噬巨噬细胞的不受控制的激活可导致发热、器官肿大和全血细胞减少。潜在的免疫紊乱既可以是遗传传播的，如FHL，也可以是获得性的，如IAHS或MAHS。在所有这些疾病中都可以发现触发感染，并且不能进行可靠的区分。已经为FHL制定了一项国际治疗方案。IAHS和MAHS的病死率也很高，如果疾病是进展性的，就有理由进行免疫调节治疗。
Hemophagocytic lymphohistiocytosis represents a spectrum of pathogenetically different diseases in which a T-cell induced, uncontrolled activation of phagocytosing macrophages may lead to fever, organomegaly, and pancytopenia. The underlying immunologic disturbance can either be genetically transmitted, Like in FHL, or acquired, as in IAHS or MAHS. Triggering infections can be found in all these diseases and do not allow a reliable differentiation. An international treatment protocol has been developed for FHL. IAHS and MAHS also have a high fatality rate, justifying immunomodulatory treatment if the disease is progressive.