La Rosée Paul
Klinik für Innere Medizin II, Abteilung Hämatologie und Internistische Onkologie, Universitätsklinikum Jena, Jena, Germany.
Hematology Am Soc Hematol Educ Program. 2015;2015:190-6. doi: 10.1182/asheducation-2015.1.190.
Treatment of hemophagocytic lymphohistiocytosis (HLH) has been developed primarily in pediatric centers, where familial HLH (FHL) is the leading cause of HLH in newborns and toddlers. The Histiocyte Society Study Group for HLH developed the HLH-94 and HLH-2004 treatment protocols, and these are frequently also used by centers treating HLH in adults (aHLH). These protocols contain etoposide, dexamethasone, and cyclosporine A; these agents all have strong activity against proliferation of cytotoxic T/NK-cells and macrophages, as well as inhibitory activity against the cytokine storm that induces, and maintains HLH. In children with predominantly hereditary disease, the HLH-94 protocol can be regarded as a "one size fits all" algorithm. HLH in adults is a much more heterogeneous syndrome requiring a more individualized approach depending on the underlying trigger, disease severity and course, as well as genetic background. Additionally, treatment in adults usually needs to be modified in the face of the preceding disease history and comorbidities. Interdisciplinary patient care with rheumatologists, gastroenterologists, neurologists, pediatricians, the transplant team, and pathologists is a prerequisite to successful treatment. The preferred approach should reflect a disease- and risk-adapted treatment that includes rigorous supportive care with continuous reassessment of sequential therapeutic measures. It should be recognized that the algorithm of HLH treatment in adults is based more on expert opinion than on extensive scientific evidence.
噬血细胞性淋巴组织细胞增生症（HLH）的治疗主要是在儿科中心开展的，在这些中心，家族性HLH（FHL）是新生儿和幼儿HLH的主要病因。组织细胞协会HLH研究小组制定了HLH - 94和HLH - 2004治疗方案，治疗成人HLH（aHLH）的中心也经常使用这些方案。这些方案包含依托泊苷、地塞米松和环孢素A；这些药物都对细胞毒性T/NK细胞和巨噬细胞的增殖具有强大的活性，同时对诱导和维持HLH的细胞因子风暴具有抑制活性。对于主要患有遗传性疾病的儿童，HLH - 94方案可被视为一种“一刀切”的算法。成人HLH是一种更为异质性的综合征，需要根据潜在诱因、疾病严重程度和病程以及遗传背景采取更个体化的方法。此外，鉴于既往病史和合并症，成人的治疗通常需要进行调整。与风湿病学家、胃肠病学家、神经学家、儿科医生、移植团队和病理学家进行跨学科的患者护理是成功治疗的先决条件。首选方法应反映一种针对疾病和风险的适应性治疗，包括严格的支持性护理以及对序贯治疗措施的持续重新评估。应该认识到，成人HLH治疗的算法更多地基于专家意见而非广泛的科学证据。
