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The presence of α‐thalassaemia trait blunts the response to hydroxycarbamide in patients with sickle cell disease
Author(s) -
Vasavda Nisha,
Badiger Sheela,
Rees David,
Height Sue,
Howard Jo,
Thein Swee Lay
Publication year - 2008
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2008.07375.x
Subject(s) - hydroxycarbamide , medicine , mean corpuscular volume , fetal hemoglobin , hemoglobinopathy , disease , beta (programming language) , clinical trial , gastroenterology , immunology , fetus , hemoglobin , biology , genetics , pregnancy , computer science , programming language
Summary 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 β‐globin locus. Approximately 30% of SCD patients have co‐inherited α‐thalassaemia resulting in hypochromic and microcytic erythrocytes. We provide data from 30 SCD patients (10 with α‐thalassaemia) demonstrating that co‐existing α‐thalassaemia significantly affects several standard measures of HC efficacy in SCD.