Adams J A, Barrett A J, Beard J, McCarthy D M
Department of Haematology, Westminster Hospital, London, UK.
Acta Haematol. 1988;79(1):33-7. doi: 10.1159/000205686.
Primary polycythaemia (PP), idiopathic myelofibrosis (MF), essential thrombocythaemia (ET) and chronic granulocytic or myeloid leukaemia (CGL) are clonal disorders of the pluripotent haemopoietic stem cells. We have studied granulocyte, megakaryocyte and erythroid progenitors from the peripheral blood of 7 patients with PP, 9 with ET, 19 with MF and 6 with CGL in order to characterise similarities and differences at the committed progenitor cell level. Spontaneous megakaryocytic and erythrocytic growth was characteristic of MF, PP and ET but was not seen in CGL. Circulating erythroid (BFU-E) and granulocyte/macrophage (CFU-GM) progenitors were markedly increased in MF and CGL, less raised in ET and closest to normal in PP. Erythropoietin-independent erythroid bursts (EIBFU-E) grew from the blood of patients with MF, PP and ET but spontaneous growth of megakaryocytes occurred in only MF and ET. These results suggest a progression of increasing abnormality from PP, where EIBFU-E occurred with relatively normal numbers of circulating progenitors, to ET where both EIBFU-E and megakaryocyte precursors regularly occur with elevated numbers of progenitors, to MF where spontaneous BFU-E, CFU-Mk and CFU-GM occur at high levels.
原发性红细胞增多症（PP）、特发性骨髓纤维化（MF）、原发性血小板增多症（ET）和慢性粒细胞性或髓性白血病（CGL）是多能造血干细胞的克隆性疾病。我们研究了7例PP患者、9例ET患者、19例MF患者和6例CGL患者外周血中的粒细胞、巨核细胞和红系祖细胞，以便在定向祖细胞水平上描述其异同。自发性巨核细胞和红细胞生长是MF、PP和ET的特征，但在CGL中未见。循环红系（BFU-E）和粒细胞/巨噬细胞（CFU-GM）祖细胞在MF和CGL中明显增加，在ET中升高程度较小，在PP中最接近正常。不依赖促红细胞生成素的红系集落（EIBFU-E）在MF、PP和ET患者的血液中生长，但仅在MF和ET中出现巨核细胞的自发生长。这些结果表明，异常程度逐渐增加，从PP（EIBFU-E出现时循环祖细胞数量相对正常）发展到ET（EIBFU-E和巨核细胞前体经常在祖细胞数量升高时出现），再到MF（自发性BFU-E、CFU-Mk和CFU-GM高水平出现）。
