Le Hô H, Barbarot N, Desrues B
Service de pneumologie, CHU de Rennes, Rennes, France.
Rev Mal Respir. 2010 Mar;27(3):257-60. doi: 10.1016/j.rmr.2010.02.005. Epub 2010 Mar 16.
The macrophage activation syndrome (MAS), also known as haemophagacytosis, is a non-specific clinical syndrome, which includes, among other things general debility, fever and hepatospleneomegaly. The most frequent pathological abnormalities are pancytopenia, hypertryglyceraemia, and hyperferrititinaemia. These clinical and biological criteria, which determine the severity of MAS, must be associated with a histological criterion - haemophagocytosis (with histiocytosis), which is usually medullary but can more rarely be splenic, hepatic or ganglionic. MAS can be primary or secondary. In the latter case, it is frequently associated with an infection of which tuberculosis is the most common.
We describe the case of a young immunocompetent patient who had disseminated tuberculosis complicated by MAS.
It is important to be aware of MAS, because its occurrence is associated with a poor prognosis and its optimal treatment, which requires a multidisciplinary approach, has not been fully defined. The optimum strategy in relation to specific aetiologies remains to be clarified.
巨噬细胞活化综合征(MAS),也称为噬血细胞增多症,是一种非特异性临床综合征,其表现包括全身虚弱、发热和肝脾肿大等。最常见的病理异常是全血细胞减少、高甘油三酯血症和高铁蛋白血症。这些决定MAS严重程度的临床和生物学标准,必须与组织学标准——噬血细胞现象(伴有组织细胞增多)相关联,该现象通常发生在骨髓,但较少发生在脾脏、肝脏或神经节。MAS可以是原发性的,也可以是继发性的。在后一种情况下,它常与感染相关,其中结核病最为常见。
我们描述了一名免疫功能正常的年轻患者,其患有播散性结核病并并发MAS。
认识到MAS很重要,因为它的发生与预后不良相关,并且其最佳治疗方法(需要多学科方法)尚未完全明确。针对特定病因的最佳策略仍有待阐明。