Peripheral blood immunophenotyping in a large cohort of patients with Shwachman-Diamond syndrome.
Pediatr Blood Cancer
; 66(5): e27597, 2019 05.
Article
em En
| MEDLINE
| ID: mdl-30604473
ABSTRACT
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.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Insuficiência Pancreática Exócrina
/
Doenças da Medula Óssea
/
Leucócitos Mononucleares
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Linfócitos B
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Imunofenotipagem
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Lipomatose
Tipo de estudo:
Etiology_studies
/
Incidence_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Adolescent
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Adult
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Child
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Child, preschool
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Female
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Humans
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Infant
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Male
Idioma:
En
Revista:
Pediatr Blood Cancer
Assunto da revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Ano de publicação:
2019
Tipo de documento:
Article
País de afiliação:
Itália