De Knop K J, Aerts N E, Ebo D G, Van Offel J F, Stevens W J, De Clerck L S
Departement Immunologie, Allergologie, Reumatologie, Universitair Ziekenhuis Antwerpen, Antwerpen, België.
Acta Clin Belg. 2011 Jan-Feb;66(1):66-9. doi: 10.2143/ACB.66.1.2062520.
We present a patient with therapy resistant multicentric reticulohistiocytosis (MRH). MRH is a rare granulomatous, multisystem disease characterised most frequently by disfiguring papulonodular skin lesions and sometimes a destructive polyarthritis, though any organ can be involved. Abnormal histiocytic reactions to an undetermined stimulus (possibly an associated mycobacterial infection, auto immune process or neoplastic process) have been proposed as an underlying mechanism. The diagnosis is confirmed by histopathology of the cutaneous nodules and/or synovial membrane by the presence of CD68-positive histiocytes and multinucleated giant cells with an eosinophilic 'ground-glass' cytoplasm. Recent studies have identified TNFalpha and other inflammatory cytokines to be highly expressed in the synovium and synovial fluid of affected joints in patients with MRH. Based on these findings, we treated our patient with infliximab in combination with methotrexate with marked improvement of morning stiffness, tender and swollen joint count, visual analogue scale and health assessment questionnaire after his third infusion. However, the nodules did not markedly resolve. When treating patients with MRH with TNFa neutralizing drugs, one has to keep the possible association with malignancy in 15-30% of cases in mind and these products should be used with caution.
我们报告了一名患有治疗抵抗性多中心网状组织细胞增多症（MRH）的患者。MRH是一种罕见的肉芽肿性多系统疾病，最常见的特征是毁容性丘疹结节性皮肤病变，有时还伴有破坏性多关节炎，尽管任何器官都可能受累。有人提出，对未确定刺激（可能是相关的分枝杆菌感染、自身免疫过程或肿瘤过程）的异常组织细胞反应是其潜在机制。通过皮肤结节和/或滑膜的组织病理学检查，发现CD68阳性组织细胞和具有嗜酸性“毛玻璃”细胞质的多核巨细胞，从而确诊。最近的研究表明肿瘤坏死因子α（TNFα）和其他炎性细胞因子在MRH患者受累关节的滑膜和滑液中高表达。基于这些发现，我们用英夫利昔单抗联合甲氨蝶呤治疗了我们的患者，在第三次输注后，晨僵、压痛和肿胀关节计数、视觉模拟评分和健康评估问卷均有明显改善。然而，结节并未明显消退。在用TNFα中和药物治疗MRH患者时，必须牢记在15%至30%的病例中可能与恶性肿瘤有关，应谨慎使用这些药物。
