Gratecos N, Foa J, Foa C, Sebahoun G, Carcassonne Y
Sem Hop. 1976;52(22-23):1383-7.
The authors report a case of congenital dyserythropoieses, type II, the diagnosis of which was made in a young 15 year old girl with anemia, sub-clinical jaundice and splenomegaly. The following three criteria were found: erythroblastic hyperplasia with a high percentage of binuclear cells, a double peripheral red cell membrane under the electron microscope and a positive Ham-Dacle test. An isotopic study showed a double process or erythropoiesis which was inefficacious and peripheral hemolysis. In connection with this case, the authors then recall the main characteristics of the three types of congenital dyserythropoiesis. The origin and consequences of the peripheral membrane excess of the erythroblasts characteristic of type II are then discussed.
作者报告了一例II型先天性红细胞生成异常病例，该病例诊断于一名15岁的年轻贫血女孩，伴有亚临床黄疸和脾肿大。发现以下三个标准：有高比例双核细胞的成红细胞增生、电子显微镜下外周红细胞双层细胞膜以及Ham-Dacle试验阳性。一项同位素研究显示存在双重过程即无效的红细胞生成和外周溶血。结合该病例，作者随后回顾了三种类型先天性红细胞生成异常的主要特征。然后讨论了II型特征性成红细胞外周膜过量的起源和后果。
