The author provides data pertinent to the modern classification of erythrocytosis, which rests on the pathogenetic principle. The difference in the pathogenesis of erythremia and secondary erythrocytoses was proved with the help of an erythroid culture and by examination of erythropoietins, which can be thus used for diagnostic purposes. Analysis of so-called "pure" erythrocytosis has shown that this term implies different patterns of erythrocytosis from the standpoint of the pathogenesis: erythremia, secondary erythrocytoses that are difficult to diagnose, and hereditary erythrocytosis associated with erythropoietin hyperproduction. There is no evidence for the existence of the "pure" erythroid leukemia and thus the author doubts this disease entity. Analysing the different patterns of secondary absolute erythrocytosis the author discusses specific problems of their pathogenesis. Attention is paid to erythrocytosis of a tobacco smoker, to the role of obesity and concomitant action of some factors on the development of hypoxic erythrocytosis. The data are presented on the new types of erythrocytosis, on the post-transplantation one, in particular, which occurs after kidney transplantation, and finally on erythrocytosis that develops occasionally in patients on hemodialysis. Attention is paid as well to relative erythrocytoses, risk factor in the development of thrombotic complications. The problems of etiologically and pathogenetically valid therapy of secondary erythrocytoses are reviewed.
作者提供了与基于发病机制原则的红细胞增多症现代分类相关的数据。真性红细胞增多症和继发性红细胞增多症在发病机制上的差异通过红系培养和促红细胞生成素检查得以证实,因此可用于诊断目的。对所谓“单纯性”红细胞增多症的分析表明,从发病机制的角度来看,该术语意味着不同类型的红细胞增多症:真性红细胞增多症、难以诊断的继发性红细胞增多症以及与促红细胞生成素过度产生相关的遗传性红细胞增多症。没有证据表明存在“单纯性”红系白血病,因此作者对这种疾病实体表示怀疑。在分析继发性绝对性红细胞增多症的不同类型时,作者讨论了其发病机制的具体问题。关注吸烟者的红细胞增多症、肥胖的作用以及某些因素对缺氧性红细胞增多症发展的协同作用。介绍了新型红细胞增多症的数据,特别是肾移植后发生的移植后红细胞增多症,最后介绍了血液透析患者偶尔出现的红细胞增多症。还关注相对性红细胞增多症,它是血栓形成并发症发展的危险因素。对继发性红细胞增多症病因学和发病机制上有效的治疗问题进行了综述。