Department of Pathology, St. John's Medical College and Hospital, Bangalore-560 034, India.
Indian J Dermatol Venereol Leprol. 2011 May-Jun;77(3):318-20. doi: 10.4103/0378-6323.79704.
Multicentric Reticulohistiocytosis (MRH) is a rare, systemic non-Langerhans cell histiocytosis (non-LCH) with prominent joint and skin manifestations. It is mostly self limiting. However, 15-30% of the cases are associated with malignancy and carry a poor prognosis. We report the case of a 42-year-old man who presented with multiple reddish-brown papules that on biopsy showed aggregates of oncocytic histiocytes with several multinucleate giant cells. Immunostains were positive for CD 68, CD 45 and were negative for S-100, CD1a. An impression of multicentric reticulohistiocytosis (MRH) was made, with the recommendation to screen for malignancy. Electron microscopy of the skin lesions showed features consistent with non-Langerhans cell histiocytosis. The patient was later diagnosed with acute myeloid leukemia at a follow-up visit several months later. Thus, it appears prudent to screen and follow-up adults with MRH, to identify an underlying malignant condition.
多发性网状组织细胞增生症（MRH）是一种罕见的、系统性的非朗格汉斯细胞组织细胞增生症（非 LCH），以关节和皮肤表现突出为特征。它大多是自限性的。然而，15-30%的病例与恶性肿瘤相关，预后不良。我们报告了一例 42 岁男性患者，他出现了多个红棕色丘疹，活检显示成簇的肥大细胞组织细胞，并有几个多核巨细胞。免疫组化染色 CD68、CD45 阳性，S-100、CD1a 阴性。诊断为多发性网状组织细胞增生症（MRH），建议筛查恶性肿瘤。皮肤病变的电子显微镜显示出非朗格汉斯细胞组织细胞增生症的特征。几个月后随访时，该患者被诊断为急性髓系白血病。因此，对 MRH 患者进行筛查和随访以确定潜在的恶性情况似乎是谨慎的。
