Jones M P, Rueda-Pedraza M E
Department of Hematology/Oncology, Walter Reed Army Medical Center, Washington, DC 20307-5001, USA.
Am J Hematol. 1997 Mar;54(3):253-7. doi: 10.1002/(sici)1096-8652(199703)54:3<253::aid-ajh13>3.0.co;2-e.
Involvement of the central nervous system by sinus histiocytosis with massive lymphadenopathy (SHML) is rare. Less than 30 cases have been reported. To the best of our knowledge, we describe the first case of an intramedullary spinal cord lesion which occurred in a 34-year-old black male, causing paraplegia. Laboratory evaluation revealed anemia, increased erythrocyte sedimentation rate, and polyclonal gammopathy. Microscopic examination of the lesion revealed lymphocytes within the cytoplasm of the histiocytic cells, a phenomenon known as emperipolesis. Immunohistochemical stains showed the large eosinophilic histiocytic cells to be positive for S-100 protein and Kp1 antigen. Special stains for fungi and acid-fast organisms were negative. There was no evidence of clonality in the lymphocytic aggregates. Glial fibrillary acidic protein was negative in the lesion, although positive in the surrounding tissue. These features confirmed the diagnosis of extranodal SHML involving the intramedullary spinal cord.
窦性组织细胞增生伴巨大淋巴结病（SHML）累及中枢神经系统较为罕见。报道的病例少于30例。据我们所知，我们描述了首例发生在一名34岁黑人男性身上的脊髓髓内病变，该病变导致了截瘫。实验室检查显示贫血、红细胞沉降率升高和多克隆丙种球蛋白病。病变的显微镜检查显示组织细胞的细胞质内有淋巴细胞，这种现象称为血细胞吞噬现象。免疫组织化学染色显示大的嗜酸性组织细胞对S-100蛋白和Kp1抗原呈阳性。真菌和抗酸菌的特殊染色为阴性。淋巴细胞聚集物中没有克隆性证据。病变中胶质纤维酸性蛋白为阴性，尽管周围组织呈阳性。这些特征证实了累及脊髓髓内的结外SHML的诊断。
