Albert A, Azgui Z, Buisine J, Ciaudo M, Fenneteau O, Fillola G, Lasserre M, Merle-Béral H, Mielot F, Raphael M
Service d'Hématologie Normale et Pathologique, Centre Régional de Transfusion Sanguine, Strasbourg, France.
Nouv Rev Fr Hematol (1978). 1992;34(6):435-41.
The clinical and laboratory features of 47 cases of macrophage activation syndrome (MAS) were reviewed in a workshop within the Groupe Français d'Hématologie cellulaire. There was no predilection for a particular age group, while common symptoms at presentation included fever, hepatic and splenic enlargement and profound depression of blood count. Examination of bone marrow aspirates allowed diagnosis to be established in almost all cases. The most characteristic sign of MAS was the presence of well differentiated macrophages without notable cytologic abnormalities but shown to be actively ingesting haematopoietic elements. Haemophagocytic syndromes generally occur in patients who develop infections in the context of preexisting immunologic abnormalities or neoplasms. In the majority of patients evolution of the disease was regressive, once spontaneously but often after antibiotic, antiparasitic and/or antiviral treatment accompanied or not by corticotherapy and/or chemotherapy. Some regressive phases were followed by more or less long term relapse, especially in the case of associated systemic lupus erythematosus. There exists at present no explanation for the occurrence of MAS, although one may remark its association with other pathologies, in particular congenital or acquired immune deficiencies and haemopathies. Several hypotheses have been proposed to explain the appearance and evolution of the disease and at present two pathways of investigation of MAS seen to merit attention: exploration of macrophages themselves and their secretion products and exploration of lymphocytes and NK cells. The current possibilities for these investigations should lead to a greater understanding of the physiopathology of MAS and it is to be hoped that a better application of appropriate therapy will enable control of its evolution.
在法国细胞血液学小组的一次研讨会上，对47例巨噬细胞活化综合征（MAS）的临床和实验室特征进行了回顾。MAS无特定年龄组偏好，常见症状包括发热、肝脾肿大和血细胞计数显著降低。几乎所有病例通过骨髓穿刺检查得以确诊。MAS最具特征性的体征是存在分化良好的巨噬细胞，其无明显细胞学异常，但显示出正在积极吞噬造血成分。噬血细胞综合征通常发生在已有免疫异常或肿瘤背景下发生感染的患者中。大多数患者病情呈退行性发展，一次是自发的，但通常是在抗生素、抗寄生虫和/或抗病毒治疗后，无论是否伴有皮质类固醇疗法和/或化疗。有些退行期之后会有或多或少的长期复发，尤其是在伴有系统性红斑狼疮的情况下。目前对于MAS的发生尚无解释，尽管可以注意到它与其他疾病有关，特别是先天性或获得性免疫缺陷和血液病。已经提出了几种假说来解释该疾病的出现和发展，目前MAS的两条研究途径似乎值得关注：对巨噬细胞本身及其分泌产物的研究以及对淋巴细胞和自然杀伤细胞的研究。目前这些研究的可能性应能使人们对MAS的病理生理学有更深入的了解，并且希望更好地应用适当的治疗方法能够控制其发展。
