Literature DB >> 18764867

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

Nisha Vasavda1, Sheela Badiger, David Rees, Sue Height, Jo Howard, Swee Lay Thein.   

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.

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Year:  2008        PMID： 18764867     DOI： 10.1111/j.1365-2141.2008.07375.x

Source DB:  PubMed          Journal:  Br J Haematol        ISSN： 0007-1048            Impact factor:   6.998


  8 in total

1.  Alpha-thalassaemia and response to hydroxyurea in sickle cell anaemia.

Authors:  Deepika S Darbari; Mehdi Nouraie; James G Taylor; Carlo Brugnara; Oswaldo Castro; Samir K Ballas
Journal:  Eur J Haematol       Date:  2014-01-30       Impact factor: 2.997

2.  Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia.

Authors:  Deepika S Darbari; Onyinye Onyekwere; Mehdi Nouraie; Caterina P Minniti; Lori Luchtman-Jones; Sohail Rana; Craig Sable; Gregory Ensing; Niti Dham; Andrew Campbell; Manuel Arteta; Mark T Gladwin; Oswaldo Castro; James G Taylor; Gregory J Kato; Victor Gordeuk
Journal:  J Pediatr       Date:  2011-09-03       Impact factor: 4.406

3.  Biological impact of α genes, β haplotypes, and G6PD activity in sickle cell anemia at baseline and with hydroxyurea.

Authors:  Françoise Bernaudin; Cécile Arnaud; Annie Kamdem; Isabelle Hau; Françoise Lelong; Ralph Epaud; Corinne Pondarré; Serge Pissard
Journal:  Blood Adv       Date:  2018-03-27

Review 4.  Differences in the clinical and genotypic presentation of sickle cell disease around the world.

Authors:  Santosh L Saraf; Robert E Molokie; Mehdi Nouraie; Craig A Sable; Lori Luchtman-Jones; Gregory J Ensing; Andrew D Campbell; Sohail R Rana; Xiao M Niu; Roberto F Machado; Mark T Gladwin; Victor R Gordeuk
Journal:  Paediatr Respir Rev       Date:  2013-11-15       Impact factor: 2.726

Review 5.  Sickle Cell Anemia and Its Phenotypes.

Authors:  Thomas N Williams; Swee Lay Thein
Journal:  Annu Rev Genomics Hum Genet       Date:  2018-04-11       Impact factor: 9.340

Review 6.  Evidence review of hydroxyurea for the prevention of sickle cell complications in low-income countries.

Authors:  Mercy Mulaku; Newton Opiyo; Jamlick Karumbi; Grace Kitonyi; Grace Thoithi; Mike English
Journal:  Arch Dis Child       Date:  2013-08-30       Impact factor: 3.791

7.  Phenotypic screening of the ReFRAME drug repurposing library to discover new drugs for treating sickle cell disease.

Authors:  Belhu Metaferia; Troy Cellmer; Emily B Dunkelberger; Quan Li; Eric R Henry; James Hofrichter; Dwayne Staton; Matthew M Hsieh; Anna K Conrey; John F Tisdale; Arnab K Chatterjee; Swee Lay Thein; William A Eaton
Journal:  Proc Natl Acad Sci U S A       Date:  2022-09-26       Impact factor: 12.779

8.  The pleiotropic effects of α-thalassemia on HbSS and HbSC sickle cell disease: Reduced erythrocyte cation co-transport activity, serum erythropoietin, and transfusion burden, do not translate into increased survival.

Authors:  John N Brewin; Amina Nardo-Marino; Sara Stuart-Smith; Sara El Hoss; Anke Hanneman; John Strouboulis; Stephan Menzel; John S Gibson; David C Rees
Journal:  Am J Hematol       Date:  2022-07-18       Impact factor: 13.265
  8 in total

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