Peripheral blood immunophenotyping in a large cohort of patients with Shwachman-Diamond syndrome.

Autor: Bezzerri V; Cystic Fibrosis Center, Azienda Ospedaliero Universitaria Ospedali Riuniti, Ancona, Italy., Vella A; Unit of Immunology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy., Gennaro GD; Department of Pathology and Diagnostics, Azienda Ospedaliera Universitaria Integrata, Verona, Italy., Ortolani R; Unit of Immunology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy., Nicolis E; Unit of Transfusion Medicine, Azienda Ospedaliera Universitaria Integrata, Verona, Italy., Cesaro S; Unit of Pediatric Hematology Oncology Azienda Ospedaliera Universitaria Integrata, Verona, Italy., Fabrizzi B; Cystic Fibrosis Center, Azienda Ospedaliero Universitaria Ospedali Riuniti, Ancona, Italy., Bronte V; Unit of Immunology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy., Corey SJ; Department of Pediatric Hematology/Oncology and Stem Cell Transplantation, Cleveland Clinic, Cleveland, Ohio., Cipolli M; Cystic Fibrosis Center, Azienda Ospedaliero Universitaria Ospedali Riuniti, Ancona, Italy.
Jazyk: angličtina
Zdroj: Pediatric blood & cancer [Pediatr Blood Cancer] 2019 May; Vol. 66 (5), pp. e27597. Date of Electronic Publication: 2019 Jan 02.
DOI: 10.1002/pbc.27597
Abstrakt: Shwachman-Diamond syndrome (SDS) is one of the more common inherited bone marrow failure syndromes, characterized by neutropenia, occasional thrombocytopenia, and anemia. Bone marrow evaluation reveals an increased number of monocytes and mature B cells along with decreased granulocytes. However, little is known about the subpopulations of peripheral blood cells, and few previous publications have been based on a small number of patients. Here, we report a comprehensive immunophenotypic analysis from a cohort of 37 SDS patients who display impairment mostly in the myeloid compartment with a deficiency also in the number of B cells and CD4/CD8 double-negative T cells.
(© 2019 Wiley Periodicals, Inc.)
Databáze: MEDLINE
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