Immunological profile in a pediatric population of patients with spherocytosis. A single-center experience

Silvio Marchesani; Letizia Sabatini; Valentina Bertaina; Olivia Marini; Michela Ambrosi; Margherita Di Mauro; Matilde Cossutta; Livia Schettini; Mariachiara Lodi; Gioacchino Andrea Rotulo; Paolo Palma; Giuseppe Palumbo; Giulia Ceglie

doi:10.1016/j.bcmd.2022.102700

Immunological profile in a pediatric population of patients with spherocytosis. A single-center experience

Blood Cells Mol Dis. 2023 Jan:98:102700. doi: 10.1016/j.bcmd.2022.102700. Epub 2022 Aug 27.

Affiliations

¹ University Department of Pediatrics, Bambino Gesù Children's Hospital, University of Rome Tor Vergata, Rome, Italy. Electronic address: silvio.marchesani@libero.it.
² University Department of Pediatrics, Bambino Gesù Children's Hospital, University of Rome Tor Vergata, Rome, Italy.
³ Department of Pediatric Hematology and Oncology, Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
⁴ Women's and Children's Health Department, Hematology-Oncology Clinic and Laboratory, University of Padova, Padova, Italy.
⁵ University Department of Pediatrics, Bambino Gesù Children's Hospital, University of Rome Tor Vergata, Rome, Italy; Clinical Immunology and Vaccinology Unit, Research Unit in Clinical Immunology and Vaccinology, Academic Department of Pediatrics, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
⁶ University Department of Pediatrics, Bambino Gesù Children's Hospital, University of Rome Tor Vergata, Rome, Italy; Department of Pediatric Hematology and Oncology, Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
⁷ Department of Pediatric Hematology and Oncology, Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy; Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.

PMID: 36055084
DOI: 10.1016/j.bcmd.2022.102700

Abstract

Spherocytosis is a hereditary disease caused by the deficiencies of different membrane proteins of red blood cells. Currently, splenectomy is the main therapeutic strategy available, although it is accompanied by an increased risk of sepsis. Several evidences have supported the hypothesis of spleen dysfunction in patients with spherocytosis that haven't yet undergone splenectomy. The aim of this study is to furtherly characterize this aspect, by describing the immune subpopulations in peripheral blood samples obtained from 41 pediatric patients with hereditary spherocytosis by flow cytometry, in order to evaluate changes in the composition of the immune populations compared to 16 healthy donors. Patients were divided in two groups: splenectomized and non-splenectomized. In the splenectomized population, data showed neutrophilic leukocytosis, thrombocytosis, increase in NK and reduction in CD4+ lymphocytes. However, we observed that most of the results obtained in the splenectomized group were found in the non-splenectomized patients as well (increase in neutrophils, in NK, reduction of CD19+, CD4+ lymphocytes and CD4+ and CD8+ naïve cells). The alterations of the immune system may be mainly due to the disease itself, regardless of splenectomy. Therefore, immunological criteria could be included in clinical phenotype assessment in order to better optimize the timing for splenectomy.

Keywords: Hemoglobinopathies; Hereditary spherocytosis; Immune system; Immunophenotype; Splenectomy.

MeSH terms

Child
Erythrocyte Count
Erythrocytes
Humans
Spherocytosis, Hereditary* / surgery
Spleen
Splenectomy