Clinical and hematological response to hydroxyurea in a patient with Hb lepore/beta-thalassemia

被引：26

作者：

Rigano, P ^{[1
]}

Manfre, L ^{[1
]}

LaGalla, R ^{[1
]}

Renda, D ^{[1
]}

Renda, MC ^{[1
]}

Calabrese, A ^{[1
]}

Calzolari, R ^{[1
]}

Maggio, A ^{[1
]}

机构：

[1] OSPED POLICLIN,DEPT RADIOL,I-90146 PALERMO,ITALY

来源：

HEMOGLOBIN | 1997年 / 21卷 / 03期

关键词：

D O I：

10.3109/03630269708997382

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

The possibility of increasing Hb F in vivo using drugs like 5-azacytidine, hydroxyurea, and butyrate has been established. However, in many cases this does not entail an increase in total hemoglobin. We report on a patient with Hb Lepore/beta-thalassemia being treated with hydroxyurea (30 mg/Kg/day) because of the presence of erythroid extramedullary masses with severe neurological abnormalities. During therapy the patient showed a remarkable improvement in neurological signs due to the reduction in extramedullary masses, a significant increase in both total hemoglobin (from 5.8 to 9.7 g/dl) and Hb F (from 4.9 g/dl to 9.1 g/dl). The marked improvement in hemoglobin level in our patient with Hb Lepore/beta-thalassemia suggests gamma-globin gene activation due to the DNA structure determined by the crossover event.

引用

页码：219 / 226

页数：8

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