Baruchel A, Schaison G
Unité d'Hématologie pédiatrique, Hôpital, Saint-Louis, Paris.
Nouv Rev Fr Hematol (1978). 1990;32(6):415-20.
The concept of inappropriate macrophage activation (IMA) embraces the clinicopathologic conditions associated with non-malignant lymphohistiocytic proliferation: familial lymphohistiocytosis (FLH) and hemophagocytic syndromes associated with viral, bacterial, fungal or parasitic infections in both immunodeficient and immunocompetent patients. IMA is also observed during the clinical course of primary immunodeficiencies (Chediak Higashi). The mechanisms of IMA are still unknown. Prognosis varies according to the immune status of the patients but is often very poor. Steroids but principally VP16-213 can slow the macrophage hyperactivation. Allogenic bone marrow grafting is indicated in FLH.
不适当巨噬细胞活化（IMA）的概念涵盖了与非恶性淋巴细胞组织细胞增生相关的临床病理情况：家族性淋巴细胞组织细胞增生症（FLH）以及免疫缺陷和免疫功能正常患者中与病毒、细菌、真菌或寄生虫感染相关的噬血细胞综合征。在原发性免疫缺陷病（切-东综合征）的临床过程中也可观察到IMA。IMA的机制仍不清楚。预后因患者的免疫状态而异，但通常很差。类固醇药物尤其是VP16-213可以减缓巨噬细胞的过度活化。FLH患者适合进行同种异体骨髓移植。
