本文报道1例遗传性口形细胞增多症。患者巩膜黄染9年、贫血、脾大、胆结石,于1983年7月5日入院。实验室检查发现网织红细胞计数增高,外周血片口形细胞计数11～40％。新鲜红细胞的钠、钾测定及脆性试验均正常,但经延长孵育(48小时)后再行测定,红细胞内钠明显增高,钾明显减少。红细胞脆性及自溶现象均增强,加葡萄糖或ATP可获部分改善。~(51)Cr红细胞寿命测定明显缩短。无G-6-PD缺陷及血红蛋白异常。对本病的发病机理、诊断及鉴别诊断进行了讨论。 This article reports 1 case of hereditary oral mytiocytosis. Scleral yellow dye patients 9 years, anemia, splenomegaly, gallstones, in July 5, 1983 admitted. Laboratory tests found that reticulocyte count increased peripheral blood lipoblast cell count 11 ~ 40%. Fresh red blood cells sodium and potassium determination and brittleness test were normal, but after prolonged incubation (48 hours) and then measured, intracellular sodium was significantly increased, potassium decreased significantly. Erythrocyte fragility and autolysis are enhanced, with glucose or ATP can be partially improved. ~ (51) Cr erythrocyte life expectancy was significantly shortened. No G-6-PD defects and hemoglobin abnormalities. The pathogenesis of the disease, diagnosis and differential diagnosis were discussed.