Clément F
Schweiz Med Wochenschr. 1981 Aug 15;111(33):1196-201.
The primary polycythemias result from malignant proliferation of myeloid stem-cell. Typically, an increase of red cell mass and a decrease of erythropoietin is found. In polycythemia vera, augmentation of PCV is frequently associated with elevation of WBC and platelets, as well as splenomegaly. The treatment consists of venosection and administration of P32 or cytostatics; all of which methods exhibit a specific risk. In secondary polycythemias, augmentation of red cell mass is consecutive to increased erythropoietin production; these hypererythropoietinemias may be induced by hypoxia or, rarely, may result from an inappropriate tumoral (malignant or benign) secretion. "Spurious" polycythemias are finally defined by the more or less normal red cell mass. They are divided into three groups: micropolycythemias, relative polycythemias and "spurious" chronic polycythemias. The latter are frequent and exhibit relatively important morbidity and mortality, and therefore the recently proposed new therapeutic approaches should be considered. Tobacco addiction appears to be one of the major causes of these "spurious" polycythemias.
原发性红细胞增多症源于髓系干细胞的恶性增殖。通常会发现红细胞量增加而促红细胞生成素减少。在真性红细胞增多症中,血细胞比容增加常伴有白细胞和血小板升高以及脾肿大。治疗方法包括静脉放血和给予P32或细胞抑制剂;所有这些方法都有特定风险。在继发性红细胞增多症中,红细胞量增加是促红细胞生成素产生增加的结果;这些红细胞生成素过多症可能由缺氧诱导,或很少情况下由不适当的肿瘤(恶性或良性)分泌引起。“假性”红细胞增多症最终由或多或少正常的红细胞量定义。它们分为三组:微小红细胞增多症、相对性红细胞增多症和“假性”慢性红细胞增多症。后者很常见,且发病率和死亡率相对较高,因此应考虑最近提出的新治疗方法。烟草成瘾似乎是这些“假性”红细胞增多症的主要原因之一。