Adachi M, Ryo R, Yoshida A, Yamaguchi N, Izumi Y
Department of Laboratory Medicine, Kobe University School of Medicine, Japan.
Acta Haematol. 1989;81(2):104-8. doi: 10.1159/000205536.
A 72-year-old man with refractory anemia (RA) developed overt megakaryoblastic leukemia after the course of RA with excess of blasts. The blasts were positive for platelet peroxidase activity and had platelet glycoproteins (GPs) such as GPIIb/IIIa and GPIIIa. The bone marrow biopsy at terminal stage disclosed marked fibrosis. The nature of the megakaryoblasts was investigated. The blasts did not differentiate morphologically into mature megakaryocytes with TPA addition. In vitro colony assay showed the failure of colony-forming unit, megakaryocyte growth in peripheral blood. The pathogenesis of myelofibrosis in our patient is discussed.
一名72岁难治性贫血(RA)男性患者,在RA伴原始细胞增多病程后发展为明显的巨核母细胞白血病。原始细胞血小板过氧化物酶活性呈阳性,且具有血小板糖蛋白(GPs),如GPIIb/IIIa和GPIIIa。终末期骨髓活检显示明显纤维化。对巨核母细胞的性质进行了研究。添加佛波酯后,原始细胞在形态上未分化为成熟巨核细胞。体外集落试验显示外周血中巨核细胞集落形成单位生长失败。对我们患者骨髓纤维化的发病机制进行了讨论。