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Hematological differences between patients with different subtypes of sickle cell disease on hydroxyurea treatment

OBJECTIVE: Sickle cell anemia and the interaction S/Beta thalassemia differ in hematological values due to microcytosis and hypochromia caused by the thalassemic mutation. The clinical benefit of long-term hydroxyurea treatment is undeniable in sickle cell disease with monitoring of the biological a...

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Detalles Bibliográficos
Autores principales: Neves, Fabia, Menezes Neto, Osvaldo Alves, Polis, Larissa Bueno, Bassi, Sarah Cristina, Brunetta, Denise Menezes, Silva-Pinto, Ana Cristina, Angulo, Ivan Lucena
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Associação Brasileira de Hematologia e Hemoterapia 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3545429/
https://www.ncbi.nlm.nih.gov/pubmed/23323066
http://dx.doi.org/10.5581/1516-8484.20120107
Descripción
Sumario:OBJECTIVE: Sickle cell anemia and the interaction S/Beta thalassemia differ in hematological values due to microcytosis and hypochromia caused by the thalassemic mutation. The clinical benefit of long-term hydroxyurea treatment is undeniable in sickle cell disease with monitoring of the biological action of the drug being by the complete blood count. The objective of this work is to compare changes in some of the erythrocytic indexes between S/Beta thalassemia and sickle cell anemia patients on long-term hydroxyurea treatment. METHODS: The values of erythrocyte indexes (mean corpuscular volume and mean corpuscular hemoglobin) were compared in a retrospective study of two groups of patients (Sickle cell anemia and S/Beta thalassemia) on hydroxyurea treatment over a mean of six years. RESULTS: The quantitative values of the two parameters differed between the groups. Increases in mean corpuscular volume and reductions in mean corpuscular hemoglobin delay longer in S/Beta thalassemia patients (p-value = 0.018). CONCLUSION: Hematological changes are some of the beneficial effects of hydroxyurea in sickle cell disease as cellular hydration increases and the hemoglobin S concentration is reduced. The complete blood count is the best test to monitor changes, but the interpretation of the results in S/Beta thalassemia should be different.