How I treat hemophagocytic lymphohistiocytosis
How I treat hemophagocytic lymphohistiocytosis
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DOI：
10.1182/blood-2011-03-278127
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发表时间：
2011-10-13
期刊：
影响因子：
20.3
通讯作者：
McClain, Kenneth L.
中科院分区：
文献类型：
--
作者：
Jordan, Michael B.;Allen, Carl E.;McClain, Kenneth L.
噬血细胞性淋巴组织细胞增生症（HLH）是一种病理性免疫激活综合征，以家族性疾病或散发性疾病的形式发生，与多种触发因素有关。这种免疫失调性疾病与血细胞减少症以及极端炎症的临床体征和症状的独特组合显著相关。及时启动免疫化疗对生存至关重要，但由于HLH的罕见性、其多变的表现以及进行诊断检测所需的时间，及时诊断可能具有挑战性。动态临床过程、治疗相关发病率高风险和疾病复发使治疗复杂化。在这里，我们回顾了HLH的临床表现和模式，并描述了我们的方法来诊断和治疗这种难以捉摸的和潜在的致命条件。（血。2011; 118（15）：4041 - 4052）
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, occurring as either a familial disorder or a sporadic condition, in association with a variety of triggers. This immune dysregulatory disorder is prominently associated with cytopenias and a unique combination of clinical signs and symptoms of extreme inflammation. Prompt initiation of immunochemotherapy is essential for survival, but timely diagnosis may be challenging because of the rarity of HLH, its variable presentation, and the time required to perform diagnostic testing. Therapy is complicated by dynamic clinical course, high risk of treatment-related morbidity, and disease recurrence. Here, we review the clinical manifestations and patterns of HLH and describe our approach to the diagnosis and therapy for this elusive and potentially lethal condition. (Blood. 2011; 118(15):4041-4052)