Mezger J, Permanetter W, Gerhartz H, Bartl R, Bauchinger M, Schmetzer H, Sauer H
Department of Internal Medicine III, Klinikum Grosshadern, University of Munich, Federal Republic of Germany.
Leuk Res. 1990;14(2):169-75. doi: 10.1016/0145-2126(90)90046-c.
We describe a patient with fever and multiple osteolytic bone lesions accompanied by hypercalcemia, a duodenal ulcer, anemia, and thrombocytopenia. Bone marrow showed a dense infiltration by abnormal cells characterized by small basophil granula, erythrophagocytosis and nuclear atypia. These cells were positive for toluidine blue and partly for myeloperoxidase and chloroacetate esterase, expressed myeloid differentiation markers, and exhibited multiple numerical and structural chromosome aberrations. Molecular genetic analysis showed no breakpoint cluster region rearrangement. Electron microscopy demonstrated granula both of basophil and mast cell type. Concluding, in this patient an acute hematopoietic malignancy with many features of malignant mastocytosis but also with signs of a basophil differentiation. This is further support for a hematopoietic stem cell origin of human mast cells.
我们描述了一名患者，其有发热、多处溶骨性骨病变，并伴有高钙血症、十二指肠溃疡、贫血和血小板减少症。骨髓显示有异常细胞的密集浸润，这些细胞的特征为小嗜碱性粒细胞颗粒、红细胞吞噬作用和核异型性。这些细胞甲苯胺蓝染色呈阳性，部分髓过氧化物酶和氯乙酸酯酶染色呈阳性，表达髓系分化标志物，并表现出多种数量和结构染色体畸变。分子遗传学分析显示无断裂点簇集区重排。电子显微镜显示有嗜碱性粒细胞型和肥大细胞型颗粒。总之，该患者患有一种急性造血系统恶性肿瘤，具有许多恶性肥大细胞增多症的特征，但也有嗜碱性粒细胞分化的迹象。这进一步支持了人类肥大细胞起源于造血干细胞的观点。
