Hematol Transfus Cell Ther. 2021 Dec 09; doi: 10.1016/j.htct.2021.08.015. Epub 2021 Dec 09.

Sickle cell anemia: hierarchical cluster analysis and clinical profile in a cohort in Brazil.

Hematology, transfusion and cell therapy

Valéria de Freitas Dutra, Thais Priscila Biassi, Maria Stella Figueiredo

PMID: 34930711 DOI: 10.1016/j.htct.2021.08.015

Abstract

INTRODUCTION: Sickle cell anemia is a monogenic disorder caused by a mutation in the β-hemoglobin gene, resulting in sickle hemoglobin that can polymerize. Presentation and clinical course have significant inter-individual variability and classifying these patients for severity is a challenge.

METHODS: We applied hierarchical clusters with 10 routine laboratory tests to understand if this grouping could be associated with clinical manifestations. We included 145 adult homozygous patients (SS) at an outpatient clinic in a retrospective study.

RESULTS: We found five clusters by counting those that had been differentiated by unconjugated bilirubin, reticulocytes, LDH, leukocytes, lymphocytes and monocytes. When comparing groups to clinical findings, the clusters were different only for liver abnormality. Cluster 3 had the lower median of reticulocytes, LDH, leukocytes, lymphocytes and monocytes and a higher percentage of patients under treatment. Clusters 4 and 5 had higher frequencies of liver impairment and higher medians of reticulocytes, LDH, leukocytes, lymphocytes and monocytes. Hemolysis and inflammation seemed to influence the grouping.

CONCLUSION: In our study, cluster analysis showed five groups that exhibited different degrees of inflammation and hemolysis. When comparing clinical data, the result was different only for the criteria of liver abnormality.

Copyright © 2021 Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular. Published by Elsevier España, S.L.U. All rights reserved.

Keywords: Hemoglobin S; Hierarchical clusters; Sickle cell anemia

Conflict of interest statement

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• Journal Article