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酸溶血试验阳性的遗传性幼红细胞多核症(HEMPAS)是以贫血、黄疸、肝脾肿大。无效红细胞造血、骨髓中有多核的幼红细胞、酸溶血试验阳性和异常的红细胞膜抗原为主要特征的常染色体隐性遗传性疾患。1951年 Wolff 报告了发生在一个家庭中的三个成员的红细胞多核症,以后陆续有类似的报告。1968年 Heimpel 总结了这些病例,认为不能归属于过去已知的疾病而称之为先天性红细胞生成不良性贫血(CDA),并根据形态学特点分为Ⅰ、Ⅱ、Ⅲ三型。1969年 Crook-
Acid hemolysis test positive hereditary erythroid polycythemia (HEMPAS) is anemia, jaundice, hepatosplenomegaly. Ineffective erythrocyte hematopoiesis, erythrocytes with multi-nucleus in bone marrow, autosomal recessive hereditary disorders characterized by positive acid hemolysis test and abnormal erythrocyte membrane antigens. Wolff, in 1951, reported erythrocyte polycythemia in three members of a family and reported similar cases later. In 1968, Heimpel summarized these cases and concluded that they should not be classified as past known diseases as congenital erythropathy (anemia) and classified according to their morphological characteristics as type I, II and III. 1969 Crook-