HETEROGENEITY OF THE ERYTHROPOIETIC DEFECT IN 2 CASES OF AASE-SMITH SYNDROME

被引：2

作者：

DAVANZO, M ^{[1
]}

PISTOIA, V ^{[1
]}

SANTINELLI, R ^{[1
]}

TOLONE, C ^{[1
]}

TORALDO, R ^{[1
]}

CORCIONE, A ^{[1
]}

CANINO, G ^{[1
]}

IAFUSCO, F ^{[1
]}

机构：

[1] IST SCI G GASLINI,ONCOL LAB,GENOA,ITALY

来源：

PEDIATRIC HEMATOLOGY AND ONCOLOGY | 1994年 / 11卷 / 02期

关键词：

AASE-SMITH-SYNDROME; ERYTHROID COLONY GROWTH;

D O I：

10.3109/08880019409141655

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Here we report two children with Aase-Smith syndrome (triphalangeal thumbs and congenital red cell plasia). In vitro growth of erythroid colonies was normal in the first patient and totally absent in the other. In both patients, treatment with glucocorticoids induced remission of anemia. Our results suggest that the different growth patterns of erythroid colonies observed in the two patients could reflect the defect of erythroid differentiation occurring at discrete maturational levels.

引用

页码：189 / 195

页数：7

共 16 条

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