Stéphan J L, Galambrun C
Unité d'hématologie et d'oncologie pédiatrique, Hôpital Nord, CHRU, Saint-Etienne, France.
Arch Pediatr. 2000 Mar;7(3):278-86. doi: 10.1016/s0929-693x(00)88746-1.
Reactive haemophagocytic syndrome is characterized by activation and uncontrolled non-malignant proliferation of T lymphocytes and macrophages, leading to a cytokine overproduction, which accounts for the main biological signs. Children usually present with an acute febrile illness, fulminant pancytopenia and hepatosplenomegaly, posing a problem of differential diagnosis with severe sepsis. Hemopoietic cells are actively ingested by monocytes/macrophages in various organs, including lymph nodes, bone marrow, liver and spleen. This exarcerbation of the histiocytic system is currently classified among the reactional histiocytoses. It reflects an inappropriate host immune response. Most patients have a known underlying disease (hemopathy, lupus, systemic juvenile arthritis, HIV infection). In the few cases that occur in the apparent absence of any risk factors, investigations should be made to look for predisposing inherited diseases, such as familial lymphohistiocytosis or Purtilo's disease in boys. The treatment rests on immunosuppressive agents, followed by bone marrow transplantation, which can provide a definitive cure in genetic forms.
反应性噬血细胞综合征的特征是T淋巴细胞和巨噬细胞活化及不受控制的非恶性增殖，导致细胞因子过度产生，这是主要的生物学征象。儿童通常表现为急性发热性疾病、暴发性全血细胞减少和肝脾肿大，这给与严重脓毒症的鉴别诊断带来问题。造血细胞在包括淋巴结、骨髓、肝脏和脾脏在内的各个器官中被单核细胞/巨噬细胞主动吞噬。这种组织细胞系统的亢进目前被归类为反应性组织细胞增多症。它反映了不适当的宿主免疫反应。大多数患者有已知的基础疾病（血液病、狼疮、系统性幼年型关节炎、HIV感染）。在少数明显没有任何危险因素的病例中，应进行检查以寻找易患的遗传性疾病，如男孩中的家族性淋巴细胞组织细胞增多症或普尔蒂洛病。治疗依赖于免疫抑制剂，随后进行骨髓移植，这可以为遗传性形式提供根治。
