Cynamon H A, Isenberg J N, Gustavson L P, Gourley W K
J Pediatr Gastroenterol Nutr. 1985 Aug;4(4):542-9. doi: 10.1097/00005176-198508000-00008.
Spur cell anemia of liver disease is a hemolytic process characterized by spiculated erythrocytes and an elevated red cell membrane cholesterol/phospholipid (C/PL) molar ratio. This form of anemia is associated almost exclusively with adults in the advanced stages of alcoholic cirrhosis. We were therefore surprised to identify two unrelated infants with cholestatic liver disease and hemolytic anemia who had spiculated erythrocytes as the major abnormal cell form on peripheral smear. Erythrocyte membrane cholesterol and phospholipid determinations from these patients were compared with six infants with extrahepatic biliary atresia and target-shaped erythrocytes and with five normal adults. Erythrocyte C/PL molar ratio distinguished target cells from normal erythrocytes (p less than 0.01). The spur cell patients' erythrocyte C/PL molar ratios were clearly greater than either target cell patients or normal controls (1.30 vs. 1.02 vs. 0.84). Both patients' spur cell anemia resolved and target cells became the major abnormal erythrocyte form. These studies identify a transient form of spur cell anemia associated with infantile cholestatic liver disease. The factors leading to the formation of spur cell anemia in infancy require further investigation.
肝病性棘状细胞贫血是一种溶血过程，其特征为红细胞呈棘状以及红细胞膜胆固醇/磷脂（C/PL）摩尔比升高。这种贫血形式几乎仅与酒精性肝硬化晚期的成年人相关。因此，我们很惊讶地发现两名患有胆汁淤积性肝病和溶血性贫血的无关婴儿，在外周血涂片上，棘状红细胞是主要的异常细胞形态。将这些患者的红细胞膜胆固醇和磷脂测定结果与六名患有肝外胆道闭锁且红细胞呈靶形的婴儿以及五名正常成年人进行了比较。红细胞C/PL摩尔比可区分靶形细胞与正常红细胞（p<0.01）。棘状细胞患者的红细胞C/PL摩尔比明显高于靶形细胞患者或正常对照组（分别为1.30、1.02和0.84）。两名患者的棘状细胞贫血均得到缓解，靶形细胞成为主要的异常红细胞形态。这些研究确定了一种与婴儿胆汁淤积性肝病相关的短暂性棘状细胞贫血形式。导致婴儿期棘状细胞贫血形成的因素需要进一步研究。
