Shibata K, Watanabe M, Yano H, Matsuzaki M, Funai N, Sano M
Department of Internal Medicine, Saga Medical School, Japan.
Haematologia (Budap). 1998;29(3):241-53.
To the significance of basophilia in haematopoietic disorders, six draw attention to cases have been analyzed. Associated diseases included acute myelogenous leukaemia (AML-M2, M3, M4, and M6), refractory anaemia with excess of blasts (RAEB) and RAEB in transformation (RAEB-T). Two AML cases (M2, M6) were preceeded by myelodysplastic syndromes (MDS). All patients showed greater than 3% basophilia in peripheral blood and bone marrow. Basophils were identified successfully by metachromatic staining with toluidine blue in all cases. Three patients (M3, M4, RAEB) presented with lymphadenopathy, suggesting an association with extramedullary involvement. Neutrophil alkaline phosphatase (NAP) activity was significantly reduced in four patients with AML (M2, M3, M4) and RAEB-T. The clinical course was generally unfavourable characterized by short remission duration or disease progression except for the patient with RAEB. Haemorrhage was the main cause of death rather than infection. Cytogenetic analysis revealed unique abnormalities involving chromosomes 3q21, 5q31, and 17q11 where the genes for some haematopoietic growth factors or their receptors are located, in addition to t(6;9) and t(15;17).
为了探讨嗜碱性粒细胞增多在造血系统疾病中的意义，分析了6例引起关注的病例。相关疾病包括急性髓系白血病（AML-M2、M3、M4和M6）、伴原始细胞增多的难治性贫血（RAEB）和转化中的RAEB（RAEB-T）。2例AML病例（M2、M6）之前有骨髓增生异常综合征（MDS）。所有患者外周血和骨髓中的嗜碱性粒细胞均大于3%。所有病例均通过甲苯胺蓝的异染性染色成功鉴定出嗜碱性粒细胞。3例患者（M3、M4、RAEB）出现淋巴结病，提示与髓外受累有关。4例AML患者（M2、M3、M4）和RAEB-T患者的中性粒细胞碱性磷酸酶（NAP）活性显著降低。除RAEB患者外，临床病程一般不佳，其特征为缓解期短或疾病进展。出血是主要死因而非感染。细胞遗传学分析显示，除t(6;9)和t(15;17)外，还存在涉及3q21、5q31和17q11染色体的独特异常，某些造血生长因子或其受体的基因位于这些染色体上。
