Bernard J F, Bournier O, Renoux M, Charron D, Boivin P
Scand J Haematol. 1976 Sep;17(3):231-9. doi: 10.1111/j.1600-0609.1976.tb01180.x.
An unclassified case of haemolytic anaemia with voluminous splenomegaly is reported. This anaemia was normocytic without any specific morphologic aspect of red blood cells (RBC); Coombs test was negative; the osmotic fragility was normal; the increased autohaemolysis was not affected by the presence of glucose; Hb studies were normal; no RBC enzyme deficiency was found; RBC lipids and membrane proteins were normal; there was a marked reduction in RBC survival with exclusive splenic uptake of erythrocytes. Before splenectomy, RBC cations and water content were abnormal: 1) the RBC water was decreased moderately; 2) the RBC sodium was about twice the normal mean with an increased 22Na turn-over; 3) the RBC potassium was markedly reduced and 42K influx was twice the normal mean; 4) the RBC calcium content was increased. Splenectomy was followed by rapid disappearance of haemolysis and RBC water and cation disturbances. Because of this extremely rapid disappearance after splenectomy the authors suggest this case of haemolytic anaemia could be a primary disease of the spleen.
报告了一例伴有巨脾的未分类溶血性贫血病例。该贫血为正细胞性，红细胞无任何特异性形态学特征；库姆斯试验阴性；渗透脆性正常；自身溶血增加不受葡萄糖存在的影响；血红蛋白研究正常；未发现红细胞酶缺乏；红细胞脂质和膜蛋白正常；红细胞存活期显著缩短，红细胞仅被脾脏摄取。脾切除术前，红细胞阳离子和水分含量异常：1）红细胞内水分适度减少；2）红细胞钠含量约为正常均值的两倍，22Na周转率增加；3）红细胞钾含量显著降低，42K内流为正常均值的两倍；4）红细胞钙含量增加。脾切除术后溶血迅速消失，红细胞水分和阳离子紊乱也随之消失。由于脾切除术后这种极其迅速的消失，作者认为该例溶血性贫血可能是脾脏的原发性疾病。
