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Influence of alpha thalassemia on clinical and laboratory parameters among nigerian children with sickle cell anemia

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Autor(es):
Olatunya, Oladele S. [1, 2] ; Albuquerque, Dulcineia M. [1] ; Adekile, Adekunle [3] ; Costa, Fernando F. [1]
Número total de Autores: 4
Afiliação do(s) autor(es):
[1] Univ Estadual Campinas, Hematol & Hemotherapy Ctr, Campinas, SP - Brazil
[2] Ekiti State Univ, Coll Med, Dept Paediat, Ado Ekiti, Ekiti State - Nigeria
[3] Kuwait Univ, Fac Med, Dept Paediat, Kuwait - Kuwait
Número total de Afiliações: 3
Tipo de documento: Artigo Científico
Fonte: JOURNAL OF CLINICAL LABORATORY ANALYSIS; v. 33, n. 2 FEB 2019.
Citações Web of Science: 0
Resumo

Background There is paucity of data on the influence of alpha thalassemia on the clinical and laboratory parameters among Nigerian sickle cell anemia (SCA) patients. This study aimed to determine the prevalence of alpha thalassemia and the influence of alpha thalassemia on laboratory parameters and clinical manifestations in a group of young Nigerian SCA patients. Methods This was a cross-sectional retrospective study conducted on 100 patients with SCA and 63 controls. The diagnosis of SCA was confirmed by DNA studies. Alpha thalassemia genotyping was performed by multiplex gap-PCR method. Laboratory parameters including complete blood count, hemoglobin quantitation, serum lactate dehydrogenase (LDH), and bilirubin were determined with standard techniques. Results Alpha thalassemia was found in 41 (41.0%) patients compared to 24 (38.1%) controls (P = 0.744), and all were due to the 3.7 kappa b alpha-globin gene deletions. Alpha thalassemia was associated with more frequent bone pain crisis, higher hemoglobin concentration, red blood cell count, and HbA(2) level among the patients. On the contrary, patients with alpha thalassemia had lower mean corpuscular volume, mean corpuscular hemoglobin, and white blood cell count (WBC) (P < 0.05). There were 6 (6.0%) patients with leg ulcers, and none of them had alpha thalassemia, P = 0.04. Conclusion This study confirms that coexistence of alpha thalassemia with SCA significantly influences both the clinical and laboratory manifestations of young Nigerian SCA patients. The coexistence of this genetic modifier is associated with increased bone pain crisis and protects against sickle leg ulcers among the patients. (AU)

Processo FAPESP: 14/00984-3 - Doenças dos glóbulos vermelhos: fisiopatologia e novas abordagens terapêuticas
Beneficiário:Fernando Ferreira Costa
Linha de fomento: Auxílio à Pesquisa - Temático