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Hydroxyurea in sickle cell disease--a study of clinico-pharmacological efficacy in the Indian haplotype.
Blood Cells Mol Dis 2009 Jan-Feb; 42(1):25-31BC

Abstract

There is clinical variability in the presentation of sickle cell disease among Indians. Vaso-occlusive crisis is common among non-tribal patients. Hydroxyurea, induces fetal hemoglobin (HbF) synthesis and reduces the clinical severity of sickle cell disease but individual patients have a variable response. This study was undertaken to investigate the efficacy and safety of hydroxyurea in Indians with severe manifestations where the beta(s) gene is linked to the Arab-Indian haplotype and is associated with higher HbF levels. Seventy-seven patients (29 adult sickle homozygous, 25 pediatric sickle homozygous, 23 adult sickle beta-thalassemia) selected for hydroxyurea therapy were evaluated for clinical, hematological, biochemical and genetic parameters and were followed for 24 months. Ninety-eight point seven percent of the sickle chromosomes were linked to the Arab-Indian haplotype, 27% of patients had associated alpha thalassemia and 65% were Xmn I +/+. Seventy-eight percent of the patients had no further crises after starting hydroxyurea. This effect was accompanied by a significant increase in HbF (p<0.001), but this increase was variable in individual cases. There was also an increase in gamma gene mRNA expression in the few cases so studied. Hemoglobin levels increased significantly (p<0.001) resulting in the cessation of blood transfusions. Leucopoenia was observed in one patient. Hydroxyurea was effective in reducing the clinical severity in Indian patients who initially had higher HbF levels and the presence of ameliorating factors, such as alpha-thalassemia and the Xmn I polymorphism. Hydroxyurea therapy with careful monitoring can thus change the quality of life of Indians with sickle cell disease.

Authors+Show Affiliations

National Institute of Immunohaematology, KEM Hospital Campus, Parel, Mumbai, India.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Controlled Clinical Trial
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

18954999

Citation

Italia, Khushnooma, et al. "Hydroxyurea in Sickle Cell Disease--a Study of Clinico-pharmacological Efficacy in the Indian Haplotype." Blood Cells, Molecules & Diseases, vol. 42, no. 1, 2009, pp. 25-31.
Italia K, Jain D, Gattani S, et al. Hydroxyurea in sickle cell disease--a study of clinico-pharmacological efficacy in the Indian haplotype. Blood Cells Mol Dis. 2009;42(1):25-31.
Italia, K., Jain, D., Gattani, S., Jijina, F., Nadkarni, A., Sawant, P., ... Colah, R. (2009). Hydroxyurea in sickle cell disease--a study of clinico-pharmacological efficacy in the Indian haplotype. Blood Cells, Molecules & Diseases, 42(1), pp. 25-31. doi:10.1016/j.bcmd.2008.08.003.
Italia K, et al. Hydroxyurea in Sickle Cell Disease--a Study of Clinico-pharmacological Efficacy in the Indian Haplotype. Blood Cells Mol Dis. 2009;42(1):25-31. PubMed PMID: 18954999.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hydroxyurea in sickle cell disease--a study of clinico-pharmacological efficacy in the Indian haplotype. AU - Italia,Khushnooma, AU - Jain,Dipty, AU - Gattani,Sushma, AU - Jijina,Farah, AU - Nadkarni,Anita, AU - Sawant,Pratibha, AU - Nair,Sona, AU - Mohanty,Dipika, AU - Ghosh,Kanjaksha, AU - Colah,Roshan, Y1 - 2008/10/26/ PY - 2008/07/31/received PY - 2008/08/14/accepted PY - 2008/10/29/pubmed PY - 2009/2/10/medline PY - 2008/10/29/entrez SP - 25 EP - 31 JF - Blood cells, molecules & diseases JO - Blood Cells Mol. Dis. VL - 42 IS - 1 N2 - There is clinical variability in the presentation of sickle cell disease among Indians. Vaso-occlusive crisis is common among non-tribal patients. Hydroxyurea, induces fetal hemoglobin (HbF) synthesis and reduces the clinical severity of sickle cell disease but individual patients have a variable response. This study was undertaken to investigate the efficacy and safety of hydroxyurea in Indians with severe manifestations where the beta(s) gene is linked to the Arab-Indian haplotype and is associated with higher HbF levels. Seventy-seven patients (29 adult sickle homozygous, 25 pediatric sickle homozygous, 23 adult sickle beta-thalassemia) selected for hydroxyurea therapy were evaluated for clinical, hematological, biochemical and genetic parameters and were followed for 24 months. Ninety-eight point seven percent of the sickle chromosomes were linked to the Arab-Indian haplotype, 27% of patients had associated alpha thalassemia and 65% were Xmn I +/+. Seventy-eight percent of the patients had no further crises after starting hydroxyurea. This effect was accompanied by a significant increase in HbF (p<0.001), but this increase was variable in individual cases. There was also an increase in gamma gene mRNA expression in the few cases so studied. Hemoglobin levels increased significantly (p<0.001) resulting in the cessation of blood transfusions. Leucopoenia was observed in one patient. Hydroxyurea was effective in reducing the clinical severity in Indian patients who initially had higher HbF levels and the presence of ameliorating factors, such as alpha-thalassemia and the Xmn I polymorphism. Hydroxyurea therapy with careful monitoring can thus change the quality of life of Indians with sickle cell disease. SN - 1096-0961 UR - https://www.unboundmedicine.com/medline/citation/18954999/Hydroxyurea_in_sickle_cell_disease__a_study_of_clinico_pharmacological_efficacy_in_the_Indian_haplotype_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1079-9796(08)00176-9 DB - PRIME DP - Unbound Medicine ER -