Primary Immunodeficiency in Children With Autoimmune Cytopenias: Retrospective 154-Patient Cohort.

Autor:	Westermann-Clark E; Division of Allergy and Immunology, Department of Pediatrics, Morsani College of Medicine, University of South Florida, Tampa, FL, United States.; Division of Allergy and Immunology, Department of Medicine, Morsani College of Medicine, University of South Florida, Tampa, FL, United States., Meehan CA; Division of Allergy and Immunology, Department of Pediatrics, Morsani College of Medicine, University of South Florida, Tampa, FL, United States., Meyer AK; Division of Allergy and Immunology, Department of Pediatrics, National Jewish Health, Denver, CO, United States.; Graduate Medical Education, University of Colorado, Denver, CO, United States., Dasso JF; Division of Allergy and Immunology, Department of Pediatrics, Morsani College of Medicine, University of South Florida, Tampa, FL, United States.; Department of Biology, University of Tampa, Tampa, FL, United States., Amre D; Division of Allergy and Immunology, Department of Pediatrics, Morsani College of Medicine, University of South Florida, Tampa, FL, United States., Ellison M; Division of Allergy and Immunology, Department of Pediatrics, Morsani College of Medicine, University of South Florida, Tampa, FL, United States., Patel B; Division of Allergy and Immunology, Department of Pediatrics, Morsani College of Medicine, University of South Florida, Tampa, FL, United States., Betensky M; Cancer and Blood Disorders Institute, Johns Hopkins All Children's Hospital, St Petersburg, FL, United States.; Division of Hematology, Department of Pediatrics Johns Hopkins All Children's Hospital, St. Petersburg, FL, United States., Hauk CI; Cancer and Blood Disorders Institute, Johns Hopkins All Children's Hospital, St Petersburg, FL, United States., Mayer J; Cancer and Blood Disorders Institute, Johns Hopkins All Children's Hospital, St Petersburg, FL, United States., Metts J; Cancer and Blood Disorders Institute, Johns Hopkins All Children's Hospital, St Petersburg, FL, United States., Leiding JW; Division of Allergy and Immunology, Department of Pediatrics, Morsani College of Medicine, University of South Florida, Tampa, FL, United States.; Division of Allergy/Immunology, Department of Pediatrics Johns Hopkins All Children's Hospital, St. Petersburg, FL, United States., Sriaroon P; Division of Allergy and Immunology, Department of Pediatrics, Morsani College of Medicine, University of South Florida, Tampa, FL, United States.; Division of Allergy/Immunology, Department of Pediatrics Johns Hopkins All Children's Hospital, St. Petersburg, FL, United States., Kumar A; Research Methodology and Biostatistics Core, Morssani College of Medicine, University of South Florida, Tampa, FL, United States., Ayala I; Cancer and Blood Disorders Institute, Johns Hopkins All Children's Hospital, St Petersburg, FL, United States.; Division of Hematology, Department of Pediatrics Johns Hopkins All Children's Hospital, St. Petersburg, FL, United States., Walter JE; Division of Allergy and Immunology, Department of Pediatrics, Morsani College of Medicine, University of South Florida, Tampa, FL, United States.; Division of Allergy/Immunology, Department of Pediatrics Johns Hopkins All Children's Hospital, St. Petersburg, FL, United States.; Division of Allergy and Immunology, Massachusetts General Hospital for Children, Boston, MA, United States.
Jazyk:	angličtina
Zdroj:	Frontiers in immunology [Front Immunol] 2021 Apr 22; Vol. 12, pp. 649182. Date of Electronic Publication: 2021 Apr 22 (Print Publication: 2021).
DOI:	10.3389/fimmu.2021.649182
Abstrakt:	Background: Primary immunodeficiency is common among patients with autoimmune cytopenia. Objective: The purpose of this study is to retrospectively identify key clinical features and biomarkers of primary immunodeficiency (PID) in pediatric patients with autoimmune cytopenias (AIC) so as to facilitate early diagnosis and targeted therapy. Methods: Electronic medical records at a pediatric tertiary care center were reviewed. We selected 154 patients with both AIC and PID (n=17), or AIC alone (n=137) for inclusion in two cohorts. Immunoglobulin levels, vaccine titers, lymphocyte subsets (T, B and NK cells), autoantibodies, clinical characteristics, and response to treatment were recorded. Results: Clinical features associated with AIC-PID included splenomegaly, short stature, and recurrent or chronic infections. PID patients were more likely to have autoimmune hemolytic anemia (AIHA) or Evans syndrome than AIC-only patients. The AIC-PID group was also distinguished by low T cells (CD3 and CD8), low immunoglobulins (IgG and IgA), and higher prevalence of autoantibodies to red blood cells, platelets or neutrophils. AIC diagnosis preceded PID diagnosis by 3 years on average, except among those with partial DiGeorge syndrome. AIC-PID patients were more likely to fail first-line treatment. Conclusions: AIC patients, especially those with Evans syndrome or AIHA, should be evaluated for PID. Lymphocyte subsets and immune globulins serve as a rapid screen for underlying PID. Early detection of patients with comorbid PID and AIC may improve treatment outcomes. Prospective studies are needed to confirm the diagnostic clues identified and to guide targeted therapy. Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. (Copyright © 2021 Westermann-Clark, Meehan, Meyer, Dasso, Amre, Ellison, Patel, Betensky, Hauk, Mayer, Metts, Leiding, Sriaroon, Kumar, Ayala and Walter.)
Databáze:	MEDLINE
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