Department of Hematology and Clinical Immunology, Kobe City Medical Center General Hospital, 4-6 Minatojima-Nakamachi, Chuo-ku, Kobe, Japan.
Int J Hematol. 2010 Jul;92(1):168-73. doi: 10.1007/s12185-010-0603-z. Epub 2010 Jun 10.
A 70-year-old male was admitted because of back pain due to peri-vertebral tumors. The histologic picture of a needle-biopsied tumor specimen showed pleomorphic large cell infiltration into the collagen fibers. On immunohistochemistry, these abnormal cells were positive for CD68, CD163 and lysozyme but negative for CD1a, 21, 30, and S100. Flow cytometric analysis also demonstrated that these cells were positive for CD13, 14, 38, 45, 56, and HLA-DR. A bone marrow aspirate showed the marked infiltration of abnormal large cells with the same surface antigens as described above. A diagnosis of HS was made. He showed monocytosis in the peripheral blood of more than 1.0 x 10(9)/L from presentation. The karyotype of bone marrow cells was 46,XY,+8. Fluorescent in situ hybridization (FISH) analysis with a probe for chromosome no. 8 showed that all these monocytes carried +8, indicating that he had another disorder of chronic myelomonocytic leukemia (CMML). FISH analysis with a probe for chromosome no. 12 demonstrated that the abnormal large cells in the bone marrow were all tetraploid, while analysis with the chromosome no. 8 probe showed more than 8 signals per cell, indicating that HS cells carried octasomy to decasomy of chromosome no. 8. These findings strongly suggest that HS in the present patient originated from underlying CMML.
一位 70 岁男性因椎旁肿瘤引起的背痛入院。经针吸活检肿瘤标本的组织学图片显示多形性大细胞浸润胶原纤维。免疫组化染色这些异常细胞 CD68、CD163 和溶菌酶阳性，但 CD1a、21、30 和 S100 阴性。流式细胞术分析也表明这些细胞 CD13、14、38、45、56 和 HLA-DR 阳性。骨髓抽吸显示异常大细胞明显浸润，表面抗原与上述相同。诊断为组织细胞肉瘤。从就诊开始，他的外周血单核细胞计数超过 1.0x10(9)/L。骨髓细胞的核型为 46,XY,+8。用 8 号染色体探针进行荧光原位杂交（FISH）分析显示所有这些单核细胞均携带+8，表明他还患有另一种慢性粒单核细胞白血病（CMML）。用 12 号染色体探针进行 FISH 分析显示骨髓中的异常大细胞均为四倍体，而用 8 号染色体探针分析显示每个细胞有超过 8 个信号，表明 HS 细胞携带 8 号染色体的八倍体至十倍体。这些发现强烈提示本患者的 HS 起源于潜在的 CMML。
