Gacon G, Wajcman H, Belkhodja-Dunda O, Labie D, Bousser J
Nouv Presse Med. 1980 Jan 26;9(5):285-9.
An abnormal hemoglobin with increased oxygen affinity has to be suspected in all the cases of polycythemia where no direct signs of "polycythemia vera", or any of the classical reasons for erythropoietic stimulation can be demonstrated. This fact is documented by two new observations, one concerning a 44 year-old man with Hb Kempsey, another concerning a 58 year-old woman with Hb Malmö. The diagnosis is based on a scrupulous electrophoretic study involving an isoelectric focusing on polyacrylamide gel, and, on the study of the oxygen binding properties of the red blood cells. This polycythemia being a compensatory mechanism allowing a normal oxygen delivery to the tissues has to be respected and a compromise must be found with the cardiovascular risk.
在所有真性红细胞增多症病例中，若未发现“真性红细胞增多症”的直接体征，或任何经典的促红细胞生成原因，就必须怀疑存在氧亲和力增加的异常血红蛋白。这一事实得到了两项新观察结果的证实，一项涉及一名患有肯普西血红蛋白的44岁男性，另一项涉及一名患有马尔默血红蛋白的58岁女性。诊断基于细致的电泳研究，包括在聚丙烯酰胺凝胶上进行等电聚焦，以及对红细胞氧结合特性的研究。这种红细胞增多症是一种代偿机制，可使组织获得正常的氧输送，必须予以重视，并且必须在心血管风险方面找到折衷办法。
