Department of Rheumatology, Fukushima Medical University School of Medicine.
Department of Neurology, Fukushima Medical University School of Medicine.
Tohoku J Exp Med. 2021 Feb;253(2):151-157. doi: 10.1620/tjem.253.151.
Mesenteric panniculitis is a chronic inflammatory disease characterized by non-specific inflammation of the adipose tissue in the mesentery. Hemophagocytic lymphohistiocytosis is a life-threating disease associated with aberrant macrophage overactivation, in which infections can be a leading cause in immunocompromised hosts. Here, we report a rare case of mesenteric panniculitis and hemophagocytic lymphohistiocytosis complicated by disseminated Mycobacterium intracellulare. A 71-year-old male with mesenteric panniculitis was admitted to our hospital for fever and pancytopenia. He was treated with oral prednisolone (15 mg/day) and cyclosporin A (150 mg/day) at presentation. Physical and laboratory examinations revealed disseminated infection with nontuberculous mycobacteria; Mycobacterium intracellulare was detected in cultures of cerebrospinal fluid, blood, sputum, and gastric fluid. Patient signs and symptoms fulfilled the five criteria for a diagnosis of hemophagocytic lymphohistiocytosis, including fever, cytopenia, hemophagocytosis, hyperferritinemia, and high soluble interleukin-2 receptor levels. Therefore, the diagnosis of nontuberculous mycobacteria-associated hemophagocytic lymphohistiocytosis was established. An anti-mycobacterial chemotherapy including chloramphenicol (800 mg/day), rifampin (450 mg/day) and ethambutol (750 mg/day) together with streptomycin (750 mg twice per week) was initiated at 30 days after admission; maintenance doses of prednisolone were increased to 60 mg/day. Fever and pancytopenia improved in response to anti-mycobacterial chemotherapy. The present case suggests that mesenteric panniculitis could be complicated with hemophagocytic lymphohistiocytosis caused by immunosuppressive therapy-associated infections as well as underlying disease activity. In conclusion, the possibility of disseminated nontuberculous mycobacteria infection with hemophagocytic lymphohistiocytosis should be considered if unexplained fever or hematological dyscrasia were presented in patients of mesenteric panniculitis.
肠系膜脂膜炎是一种以肠系膜脂肪组织非特异性炎症为特征的慢性炎症性疾病。噬血细胞性淋巴组织细胞增生症是一种危及生命的疾病，与异常的巨噬细胞过度激活有关，在免疫功能低下的宿主中，感染可能是一个主要原因。在这里，我们报告一例罕见的肠系膜脂膜炎和噬血细胞性淋巴组织细胞增生症并发播散性细胞内分枝杆菌感染的病例。一名 71 岁男性因肠系膜脂膜炎入院，表现为发热和全血细胞减少。他在就诊时接受了口服泼尼松龙（15mg/天）和环孢素 A（150mg/天）治疗。体格检查和实验室检查显示播散性非结核分枝杆菌感染；在脑脊液、血液、痰液和胃液培养中均检测到细胞内分枝杆菌。患者的症状和体征符合噬血细胞性淋巴组织细胞增生症的五项诊断标准，包括发热、血细胞减少、噬血现象、高铁蛋白血症和高可溶性白细胞介素-2 受体水平。因此，诊断为非结核分枝杆菌相关性噬血细胞性淋巴组织细胞增生症。入院后 30 天开始使用含氯霉素（800mg/天）、利福平（450mg/天）和乙胺丁醇（750mg/天）的抗分枝杆菌化疗，以及每周两次 750mg 链霉素；泼尼松龙的维持剂量增加到 60mg/天。发热和全血细胞减少对抗分枝杆菌化疗有反应。本病例提示，肠系膜脂膜炎可能与免疫抑制治疗相关感染以及基础疾病活动引起的噬血细胞性淋巴组织细胞增生症并发。总之，如果肠系膜脂膜炎患者出现不明原因发热或血液系统异常，应考虑播散性非结核分枝杆菌感染伴噬血细胞性淋巴组织细胞增生症的可能性。
