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Int. J. Mol. Sci. 2017, 18(12), 2707; https://doi.org/10.3390/ijms18122707

Cut-Off Values of Hematologic Parameters to Predict the Number of Alpha Genes Deleted in Subjects with Deletional Alpha Thalassemia

1
Servicio de Hematología, Hospital Universitario, Fundación Jiménez Díaz, IIS-FJD, Universidad Autónoma de Madrid, 28029 Madrid, Spain
2
Servicio de Hematología, Hospitales Quirón Públicos, IIS-FJD, Universidad Autónoma de Madrid, 28029 Madrid, Spain
*
Author to whom correspondence should be addressed.
Received: 19 September 2017 / Revised: 2 December 2017 / Accepted: 9 December 2017 / Published: 13 December 2017
(This article belongs to the Special Issue Thalassemia in 2017)
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Abstract

Most α-thalassemia cases are caused by deletions of the structural α-globin genes. The degree of microcytosis and hypochromia has been correlated with the number of affected α-globin genes, suggesting a promising role of hematologic parameters as predictive diagnostic tools. However, cut-off points for these parameters to discriminate between the different subtypes of α-thalassemia are yet to be clearly defined. Six hematologic parameters (RBC, Hb, MCV, MCH, MCHC and RDW) were evaluated in 129 cases of deletional α-thalassemia (56 heterozygous α+ thalassemia, 36 homozygous α+ thalassemia, 29 heterozygous α0 thalassemia and 8 cases of Hb H disease). A good correlation between the number of deleted alpha genes and MCV (r = −0.672, p < 0.001), MCH (r = −0.788, p < 0.001) and RDW (r = 0.633, p < 0.001) was observed. The presence of an α0 allele should be discarded in individuals with microcytosis without iron deficiency and normal values of Hb A2 and Hb F with MCH < 23.40 pg. Furthermore, MCH < 21.90 pg and/or MCV < 70.80 fL are strongly suggestive of the presence of one α0 allele. Finally, an accurate presumptive diagnosis of Hb H disease can be made if both RDW ≥ 20% and MCH < 19 pg are seen. View Full-Text
Keywords: alpha; thalassemia; deletional; cut-off; number of genes; microcytic anemia; differential diagnosis alpha; thalassemia; deletional; cut-off; number of genes; microcytic anemia; differential diagnosis
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Velasco-Rodríguez, D.; Blas, C.; Alonso-Domínguez, J.-M.; Vega, G.; Soto, C.; García-Raso, A.; Llamas-Sillero, P. Cut-Off Values of Hematologic Parameters to Predict the Number of Alpha Genes Deleted in Subjects with Deletional Alpha Thalassemia. Int. J. Mol. Sci. 2017, 18, 2707.

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