地中海贫血中CD177（PRV1）表达增加及潜在的红细胞生成活性。

Increased CD177 (PRV1) expression in thalassaemia and the underlying erythropoietic activity.

作者信息

Zoi Katerina, Terpos Evangelos, Zoi Christine, Loukopoulos Dimitris

机构信息

Haematology Research Laboratory, Biomedical Research Foundation, Academy of Athens, Athens, Greece.

出版信息

Br J Haematol. 2008 Apr;141(1):100-4. doi: 10.1111/j.1365-2141.2008.06993.x.

DOI:10.1111/j.1365-2141.2008.06993.x

PMID:18324972

Abstract

CD177 (PRV1) expression is strongly related to polycythaemia vera (PV). Whilst studying CD177 expression in PV patients and controls, individuals with beta-thalassaemia minor were found to display an elevated expression of CD177. The study was expanded to include patients with thalassaemia intermedia, sickle cell/beta-thalassaemia and thalassaemia major. CD177 expression was increased in these thalassaemic groups and correlated with their erythropoietic activity, as assessed by the measurement of serum erythropoietin and soluble transferrin receptor levels. Within this context, elevated CD177 expression is not only a specific feature of PV but may be an indicator of increased erythropoietic activity in thalassaemia syndromes.

摘要

CD177（PRV1）的表达与真性红细胞增多症（PV）密切相关。在研究PV患者和对照组的CD177表达时，发现轻度β地中海贫血患者的CD177表达升高。该研究进一步扩展至中间型地中海贫血、镰状细胞/β地中海贫血和重型地中海贫血患者。这些地中海贫血组的CD177表达均增加，且与通过检测血清促红细胞生成素和可溶性转铁蛋白受体水平评估的红细胞生成活性相关。在此背景下，CD177表达升高不仅是PV的一个特异性特征，还可能是地中海贫血综合征中红细胞生成活性增加的一个指标。