Mikami Makoto, Sadahira Yoshito, Suetsugu Yoshimasa, Wada Hideho, Sugihara Takashi
Department of Medicine, Kawasaki Medical School, Kurashiki, Japan.
Int J Hematol. 2004 Nov;80(4):365-9. doi: 10.1532/ijh97.04064.
We report a case of a very rare disorder, histiocytic sarcoma, from a review of our autopsy cases. The neoplastic cells that proliferated in organs throughout the body were large cells containing eosinophilic cytoplasm and pleomorphic nuclei with prominent nucleoli. In the bone marrow, erythrophagocytosis by neoplastic cells was observed. The neoplastic cells were positive not only for lysozymes and CD68 (KP-1, PG-M1, and Ki-M1P) but also for a monocyte/macrophage-specific marker, CD163. In contrast, the results of tests for markers of myeloid cells, lymphoid cells, and epithelial cells were all negative. In a polymerase chain reaction study of paraffin-embedded tissues, analyses for the rearrangement of immunoglobulin heavy chain and T-cell receptor-gamma genes were negative. The current World Health Organization diagnostic criteria for histiocytic sarcoma regard immunohistochemical investigation as crucial. In this regard, the highly specific positivity for CD163 in this patient indicates that immunohistochemical staining of CD163 is very useful for the diagnosis of histiocytic sarcoma.
通过对我们尸检病例的回顾，我们报告了一例非常罕见的疾病——组织细胞肉瘤。在全身各器官中增殖的肿瘤细胞为大细胞，含有嗜酸性细胞质和具有明显核仁的多形性细胞核。在骨髓中，观察到肿瘤细胞吞噬红细胞。肿瘤细胞不仅对溶菌酶和CD68（KP-1、PG-M1和Ki-M1P）呈阳性，而且对单核细胞/巨噬细胞特异性标志物CD163也呈阳性。相比之下，髓系细胞、淋巴细胞和上皮细胞标志物的检测结果均为阴性。在对石蜡包埋组织进行的聚合酶链反应研究中，免疫球蛋白重链和T细胞受体γ基因重排分析均为阴性。世界卫生组织目前的组织细胞肉瘤诊断标准认为免疫组织化学检查至关重要。在这方面，该患者CD163的高度特异性阳性表明，CD163免疫组织化学染色对组织细胞肉瘤的诊断非常有用。
