Honma K, Nemoto K, Ohnishi Y, Kimura K
Second Department of Pathology, Niigata University School of Medicine, Japan.
Acta Pathol Jpn. 1989 Oct;39(10):670-6. doi: 10.1111/j.1440-1827.1989.tb02415.x.
A case of blastic transformation of essential thrombocythemia (ET) is reported. A 69-year-old male was first admitted to hospital because of fever in February, 1982. He was diagnosed as having ET because of marked thrombocytosis (205.5 x 10(10)/1), absence of erythrocytosis, absence of splenomegaly, normal karyotype and no increment of blasts in the bone marrow, and normal levels of neutrophil alkaline phosphatase, vitamin B12 and folate. He was treated with busulfan, and subsequently his platelet count was well controlled for about five years. At the second admission, blasts were present in the peripheral blood, and later accounted for 49% of the total leukocyte count. Histological examination of a bone biopsy specimen showed homogeneous proliferation of blastic cells and slight reticulin fibrosis. At autopsy, the degree of bone marrow fibrosis had increased. This was considered to be a very rare case of ET with blastic transformation in the terminal phase.
报告了一例原发性血小板增多症（ET）急变的病例。一名69岁男性于1982年2月因发热首次入院。因其血小板显著增多（205.5×10¹⁰/L）、无红细胞增多、无脾肿大、核型正常、骨髓中原始细胞无增加以及中性粒细胞碱性磷酸酶、维生素B₁₂和叶酸水平正常，被诊断为ET。他接受了白消安治疗，随后其血小板计数得到了约五年的良好控制。第二次入院时，外周血中出现了原始细胞，后来占白细胞总数的49%。骨髓活检标本的组织学检查显示原始细胞均匀增生和轻度网状纤维纤维化。尸检时，骨髓纤维化程度加重。这被认为是ET终末期急变的极罕见病例。
