HEREDITARY ERYTHROBLASTIC MULTINUCLEARITY ASSOCIATED WITH A POSITIVE ACIDIFIED-SERUM TEST - A TYPE OF CONGENITAL DYSERYTHROPOIETIC ANAEMIA
HEREDITARY ERYTHROBLASTIC MULTINUCLEARITY ASSOCIATED WITH A POSITIVE ACIDIFIED-SERUM TEST - A TYPE OF CONGENITAL DYSERYTHROPOIETIC ANAEMIA
复制标题
DOI：
10.1111/j.1365-2141.1969.tb05660.x
复制
发表时间：
1969-01-01
影响因子：
6.5
通讯作者：
LEWIS, SM
中科院分区：
文献类型：
--
作者：
CROOKSTON, JH;CROOKSTON, MC;LEWIS, SM
摘要在5例患者（包括2名姐妹篇）中，一种不寻常的贫血的特征是成红细胞多核性、无效红细胞生成和酸化血清试验阳性。与PNH不同，这些患者的糖水试验总是阴性。他们的细胞被酸化的正常血清溶解，表明他们对一些正常受试者中存在的凝集和补体结合抗体的异常敏感性。患者细胞与抗-i的凝集评分较高，并且对抗-i和抗-I的裂解异常敏感。这种疾病显然是作为一种常染色体隐性遗传性状遗传的。
SummaryIn five patients (including two sisters) an unusual anaemia was characterized by erythroblastic multinuclearity, ineffective erythropoiesis and a positive acidified‐serum test. Unlike PNH, the sugar‐water test was always negative in these patients. The lysis of their cells by acidified normal sera indicated their abnormal sensitivity to an agglutinating and complement‐binding antibody present in some normal subjects. The patients cells gave high agglutination scores with anti‐i, and were unusually susceptible to lysis by anti‐i and anti‐I. The disorder is apparently inherited as an autosomal recessive character.