Stéphan J L
Unité d'hématologie et oncologie pédiatrique, hôpital NORD, CHU de Saint-Etienne, 42055 Saint-Etienne, France.
Arch Pediatr. 2003 Sep;10 Suppl 4:517s-520s. doi: 10.1016/s0929-693x(03)90060-1.
Lymphohistiocytic activation syndromes are characterized by activation and proliferation of T cells and macrophages usually reflecting an inappropriate response of the host to an infection. The clinical manifestations are often alarming symptoms suggestive of severe sepsis. Most patients have a known underlying disease (hemopathy, lupus, Still's disease, HIV infection, bone marrow or organ transplantation). In the few cases that occur apparently without any risk factors, investigations should be done to look for a predisposing inherited disease such as familial lymphohistiocytosis or Purtilo syndrome in boys. The treatment relies on immunosuppressive agents followed by bone marrow transplantation, which can provide a definitive cure in genetics forms.
淋巴细胞组织细胞活化综合征的特征是T细胞和巨噬细胞的活化与增殖，通常反映宿主对感染的不适当反应。临床表现往往是提示严重脓毒症的警示症状。大多数患者有已知的基础疾病（血液病、狼疮、斯蒂尔病、HIV感染、骨髓或器官移植）。在少数明显无任何危险因素的病例中，应进行检查以寻找潜在的遗传性疾病，如男孩的家族性淋巴细胞组织细胞增多症或普尔蒂洛综合征。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方法。
