Ohno T, Sugiyama T, Furukawa H, Ishibashi T, Okuma M
Department of Internal Medicine, Ohtsu Red Cross Hospital, Shiga, Japan.
Am J Hematol. 1994 Mar;45(3):244-7. doi: 10.1002/ajh.2830450310.
We describe a patient with malignant histiocytosis whose serum contained an autoantibody against platelet protein. The patient was admitted because of nasal bleeding and high-grade fever. The clinical course was fulminantly progressive and terminated with cerebral hemorrhage. Bone marrow aspirate showed the proliferation of large atypical cells, some of which exhibited phagocytic activity. At postmortem examination, there was diffuse infiltration of these atypical cells in the liver, spleen, kidney, and bone marrow. Morphological, cytochemical, immunohistochemical, and genotypic characteristics suggested that the proliferating cells were derived from the monocyte-macrophage system. Western blot analysis revealed the presence of autoantibody against an approximately 88 kd molecule of platelet proteins. Although the relationship of cause and effect remains to be clarified, this autoantibody appeared to have stimulated thrombophagocytosis of the neoplastic cells.
我们描述了一名患有恶性组织细胞增多症的患者，其血清中含有一种针对血小板蛋白的自身抗体。该患者因鼻出血和高热入院。临床病程呈暴发性进展，最终因脑出血死亡。骨髓穿刺显示有大量非典型细胞增殖，其中一些细胞具有吞噬活性。尸检时，这些非典型细胞在肝脏、脾脏、肾脏和骨髓中呈弥漫性浸润。形态学、细胞化学、免疫组织化学和基因特征表明，增殖细胞来源于单核细胞-巨噬细胞系统。蛋白质印迹分析显示存在针对一种约88kd血小板蛋白分子的自身抗体。尽管因果关系仍有待阐明，但这种自身抗体似乎刺激了肿瘤细胞的血小板吞噬作用。
