Department C of Hepato-gastroenterology, Ibn Sina University Hospital, Mohammed V University, Rabat, Morocco.
Pan Afr Med J. 2022 Jun 6;42:97. doi: 10.11604/pamj.2022.42.97.29361. eCollection 2022.
The macrophage activation syndrome (MAS) is a rare but potentially fatal disease. We report two cases of Crohn´s disease, one under 5-aminosalicylic acid and the other under corticosteroid, having developed MAS. The first was a male patient hospitalized for high fever, clinical examination found fever and left inguinal lymph node. The second case was a female patient hospitalized for asthenia, clinical examination found fever and oral mycosis. The biology tests revealed bi/pancytopenia, hyperferritinemia, hypofibrinogenemia. The myelogram showed hemophagocytosis. The identified infectious cause was Klebsiella pneumoniae in the urinary tract in one patient and oral Candida albicans in the other one. Thoraco-abdomino-pelvic computed tomography (CT) scan showed a thickening of the bladder in the male patient, and eliminated a deep infection and tumour cause in the female patient. The diagnosis of MAS was made, and both patients were placed on broad-spectrum antibiotic, in addition to local antifungal treatment and corticosteroid for the female patient, but the evolution was fatal for both. In conclusion, the management of MAS should be fast and multidisciplinary, based on treatment of the causal infectious agent, immunomodulatory treatment of haemophagocytosis, symptomatic treatment and replacement of organ failure.
巨噬细胞活化综合征（MAS）是一种罕见但潜在致命的疾病。我们报告了两例克罗恩病患者，一例在 5-氨基水杨酸治疗下，另一例在皮质类固醇治疗下发生 MAS。第一例是一名男性患者，因高热住院，临床检查发现发热和左侧腹股沟淋巴结肿大。第二例是一名女性患者，因乏力住院，临床检查发现发热和口腔真菌感染。生物学检查显示全血细胞减少、铁蛋白血症、纤维蛋白原血症。骨髓检查显示噬血细胞现象。确定的感染原因是一例患者的尿路感染中产酸克雷伯菌，另一例患者的口腔白色念珠菌感染。胸腹部盆腔计算机断层扫描（CT）显示男性患者的膀胱增厚，女性患者排除了深部感染和肿瘤的原因。诊断为 MAS，两名患者均接受广谱抗生素治疗，女性患者还接受局部抗真菌治疗和皮质类固醇治疗，但两名患者的病情均致命。总之，MAS 的治疗应快速且多学科，基于对病因感染的治疗、噬血细胞的免疫调节治疗、症状治疗和器官衰竭的替代治疗。
