Revista Brasileira de Hematologia e Hemoterapia Revista Brasileira de Hematologia e Hemoterapia
Rev Bras Hematol Hemoter 2017;39:140-5 DOI: 10.1016/j.bjhh.2017.02.007
Original article
Telomere length correlates with disease severity and inflammation in sickle cell disease
Marina Pereira Colellaa, Barbara A. Santanab,c, Nicola Conrana, Vinicius Tomazinia, Fernando F. Costaa, Rodrigo T. Caladob,c, Sara T. Olalla Saada,,
a Universidade Estadual de Campinas/Hemocentro (UNICAMP), Campinas, SP, Brazil
b Universidade de São Paulo (USP), Ribeirão Preto, SP, Brazil
c Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP), Ribeirão Preto, São Paulo, SP, Brazil
Recebido 17 Janeiro 2017, Aceitaram 15 Fevereiro 2017
Relacionado:
Rev Bras Hematol Hemoter 2017;39:98-9
Abstract
Background

Telomeres, the ends of linear chromosomes, shorten during mitotic cell division and erosion may be aggravated by inflammation or proliferative and oxidative stress. As the bone marrow is under hyperproliferative pressure in sickle cell disease and several tissues are submitted to chronic inflammation, this study sought to determine the telomere length of patients with sickle cell disease.

Methods

The mean telomere length was measured in peripheral blood leukocytes by quantitative polymerase chain reaction. The age-adjusted telomere to single copy gene ratio was compared between 91 adult sickle cell disease patients and 188 controls.

Results

Sickle cell disease patients had significantly shorter telomeres than the controls (p-value<0.0001). Moreover, among sickle cell disease genotypes, Hb SS patients had significantly shorter telomeres compared to Hb SC and Hb Sβ patients (p-value<0.0001). Patients on hydroxyurea also had shorter telomeres in comparison to those off the drug (p-value=0.02). A positive correlation was observed between telomere length and hemoglobin level (r=0.3; p-value=0.004), whereas negative correlations were detected between telomere length and lymphocyte count (r=−0.3; p-value=0.005) and interleukin-8 serum levels (r=−0.4; p-value=0.02).

Conclusions

The findings of this study indicate that telomeres are short in sickle cell disease patients and that telomere erosion directly correlates with disease genotype, inflammation markers, and the use of hydroxyurea.

Keywords
Sickle cell disease, Telomere length, Inflammation
Rev Bras Hematol Hemoter 2017;39:140-5 DOI: 10.1016/j.bjhh.2017.02.007