Publications

Authors:

Bezzerri, Valentino; Vella, Antonio; Gennaro, Gianfranco Di; Ortolani, Riccardo; Nicolis, Elena; Cesaro, Simone; Fabrizzi, Benedetta; Bronte, Vincenzo; Corey, Seth J; Cipolli, Marco

Title:

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

Year:

2019

Type of item:

Articolo in Rivista

Tipologia ANVUR:

Articolo su rivista

Language:

Inglese

Referee:

No

Name of journal:

PEDIATRIC BLOOD & CANCER

ISSN of journal:

1545-5009

N° Volume:

66

Number or Folder:

5

Page numbers:

27597-27601

Keyword:

Shwachman-Diamond syndrome; bone marrow failure; double-negative T cells; immunophenotype; neutropenia

Short description of contents:

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.

Product ID:

109263

Handle IRIS:

11562/997268

Last Modified:

November 15, 2022

Bibliographic citation:

Bezzerri, Valentino; Vella, Antonio; Gennaro, Gianfranco Di; Ortolani, Riccardo; Nicolis, Elena; Cesaro, Simone; Fabrizzi, Benedetta; Bronte, Vincenzo; Corey, Seth J; Cipolli, Marco, Peripheral blood immunophenotyping in a large cohort of patients with Shwachman-Diamond syndrome «PEDIATRIC BLOOD & CANCER» , vol. 66 , n. 5 , 2019 , pp. 27597-27601

Consulta la scheda completa presente nel repository istituzionale della Ricerca di Ateneo