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

Authors :
Fernando Ferreira Costa
Adekunle Adekile
Oladele Simeon Olatunya
Dulcineia M. Albuquerque
Source :
Journal of Clinical Laboratory Analysis. 33:e22656
Publication Year :
2018
Publisher :
Wiley, 2018.

Abstract

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 κb α-globin gene deletions. Alpha thalassemia was associated with more frequent bone pain crisis, higher hemoglobin concentration, red blood cell count, and HbA2 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.

Details

ISSN :
08878013
Volume :
33
Database :
OpenAIRE
Journal :
Journal of Clinical Laboratory Analysis
Accession number :
edsair.doi.dedup.....1f8403d816e67e5557315bf1755b0305
Full Text :
https://doi.org/10.1002/jcla.22656