Format

Send to:

Choose Destination
See comment in PubMed Commons below
Br J Haematol. 2008 Nov;143(4):589-92. doi: 10.1111/j.1365-2141.2008.07375.x. Epub 2008 Sep 1.

The presence of alpha-thalassaemia trait blunts the response to hydroxycarbamide in patients with sickle cell disease.

Author information

  • 1Molecular Haematology, Division of Gene and Cell Based Therapy, King's College London School of Medicine, London, UK.

Abstract

Hydroxycarbamide (HC), although a key drug therapy in sickle cell disease (SCD), does not result in a clinical response in all patients. Increases in fetal haemoglobin (HbF) and mean corpuscular volume of erythrocytes are standard clinical measures of HC efficacy in SCD. Genetic studies have determined that the majority of HbF regulation occurs outside the beta-globin locus. Approximately 30% of SCD patients have co-inherited alpha-thalassaemia resulting in hypochromic and microcytic erythrocytes. We provide data from 30 SCD patients (10 with alpha-thalassaemia) demonstrating that co-existing alpha-thalassaemia significantly affects several standard measures of HC efficacy in SCD.

PMID:
18764867
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Blackwell Publishing
    Loading ...
    Write to the Help Desk