Details

Autor(en) / Beteiligte

• da Guarda, Caroline Conceição
• Yahouédéhou, Sètondji Cocou Modeste Alexandre
• Santiago, Rayra Pereira
• Neres, Joelma Santana Dos Santos
• Fernandes, Camila Felix de Lima
• Aleluia, Milena Magalhães
• Figueiredo, Camylla Vilas Boas
• Fiuza, Luciana Magalhães
• Carvalho, Suellen Pinheiro
• Oliveira, Rodrigo Mota de
• Fonseca, Cleverson Alves
• Ndidi, Uche Samuel
• Nascimento, Valma Maria Lopes
• Rocha, Larissa Carneiro
• Goncalves, Marilda Souza
• Hodges, Mary Hamer

Titel

Sickle cell disease: A distinction of two most frequent genotypes (HbSS and HbSC)

Ist Teil von

• PloS one, 2020-01, Vol.15 (1), p.e0228399-e0228399

Ort / Verlag

United States: Public Library of Science

Erscheinungsjahr

2020

Quelle

MEDLINE

Beschreibungen/Notizen

• 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 hematological, inflammatory, hemolytic, endothelial dysfunction and anemia biomarkers in HbSC disease 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.