Sickle cell disease: A distinction of two most frequent genotypes (HbSS and HbSC)
Loading...
Date
2020-01-29
Journal Title
Journal ISSN
Volume Title
Publisher
PLoS ONE
Abstract
Sickle cell disease (SCD) consists of a group of hemoglobinopathies in which individuals
present highly variable clinical manifestations. Sickle cell anemia (SCA) is the most severe
form, while SC hemoglobinopathy (HbSC) is thought to be milder. Thus, we investigated the
clinical manifestations and laboratory parameters by comparing each SCD genotype. We
designed a cross-sectional study including 126 SCA individuals and 55 HbSC individuals in
steady-state. Hematological, biochemical and inflammatory characterization was performed
as well as investigation of previous history of clinical events. SCA patients exhibited most
prominent anemia, hemolysis, leukocytosis and inflammation, whereas HbSC patients had
increased lipid determinations. The main cause of hospitalization was pain crises on both
genotypes. Vaso-occlusive events and pain crises were associated with hematological,
inflammatory and anemia biomarkers on both groups. Cluster analysis reveals hematologi cal, inflammatory, hemolytic, endothelial dysfunction and anemia biomarkers in HbSC dis ease as well as SCA. The results found herein corroborate with previous studies suggesting
that SCA and HbSC, although may be similar from the genetic point of view, exhibit different
clinical manifestations and laboratory alterations which are useful to monitor the clinical
course of each genotype.
Description
Keywords
Joelma Santana dos Santos Neres, Ahmadu Bello University, ACENTDFB, Sickle cell anemia, hemolysis, leukocytosis
Citation
da Guarda CC, Yahoue´de´hou SCMA, Santiago RP, Neres JSdS, Fernandes CFdL, Aleluia MM, et al. (2020) Sickle cell disease: A distinction of two most frequent genotypes (HbSS and HbSC). PLoS ONE 15(1): e0228399. https://doi.org/ 10.1371/journal.pone.0228399