Oka K, Mori N, Yatabe Y, Kojima M
Section of Clinical Laboratory, Hospital, National Institute of Radiological Sciences, Chiba, Japan.
Arch Pathol Lab Med. 1992 Nov;116(11):1228-33.
Three cases of malignant histiocytosis were immunohistochemically studied. The cases included the following three patients: a 38-year-old man, a 44-year-old man, and a girl aged 5 years 9 months. All three patients died within 3 months of hospitalization. They had a high fever (temperature over 38.5 degrees C), lymph node swelling, hepatosplenomegaly, and pancytopenia. Blastoid and hemophagocytic cells proliferated in the bone marrow and lymph nodes, especially in the sinuses of the latter. We diagnosed malignant histiocytosis in the three cases based on clinical features, extremely poor prognoses, and the morphologic features and growth pattern of blastoid and hemophagocytic cells. Blastoid and hemophagocytic cells expressed phenotype Mac-387+/KP1+/lysozyme+/polyclonal CD3+. The Mac-387 and KP1 antigens and lysozyme are markers for monocytes/macrophages, and polyclonal CD3 is a marker for T lymphocytes. Therefore, we suggest that a certain number of cases of malignant histiocytosis have a biphenotypic nature, namely, the T cell and macrophage, although many cases of malignant histiocytosis have been reported as expressing only T-lymphocyte antigens.
对三例恶性组织细胞增多症进行了免疫组织化学研究。病例包括以下三名患者：一名38岁男性、一名44岁男性和一名5岁9个月的女孩。所有三名患者均在住院后3个月内死亡。他们均有高热（体温超过38.5摄氏度）、淋巴结肿大、肝脾肿大和全血细胞减少。原始样细胞和噬血细胞在骨髓和淋巴结中增殖，尤其在淋巴结的窦中。根据临床特征、极差的预后以及原始样细胞和噬血细胞的形态学特征及生长模式，我们诊断这三例为恶性组织细胞增多症。原始样细胞和噬血细胞表达表型Mac-387+/KP1+/溶菌酶+/多克隆CD3+。Mac-387和KP1抗原以及溶菌酶是单核细胞/巨噬细胞的标志物，多克隆CD3是T淋巴细胞的标志物。因此，我们认为尽管许多恶性组织细胞增多症病例据报道仅表达T淋巴细胞抗原，但一定数量的恶性组织细胞增多症病例具有双表型性质，即T细胞和巨噬细胞表型。
