Immunophenotypic analysis of hematopoiesis in patients suffering from Shwachman-Bodian-Diamond Syndrome

Angela Mercuri; Elisa Cannata; Omar Perbellini; Chiara Cugno; Rita Balter; Ada Zaccaron; Gloria Tridello; Giovanni Pizzolo; Massimiliano De Bortoli; Mauro Krampera; Marco Cipolli; Simone Cesaro

doi:10.1111/ejh.12490

Immunophenotypic analysis of hematopoiesis in patients suffering from Shwachman-Bodian-Diamond Syndrome

Angela Mercuri, Elisa Cannata, Omar Perbellini, Chiara Cugno, Rita Balter, Ada Zaccaron, Gloria Tridello, Giovanni Pizzolo, Massimiliano De Bortoli, Mauro Krampera, Marco Cipolli, Simone Cesaro

IRCCS Fondazione Policlinico San Matteo

Research output: Contribution to journal › Article › peer-review

Abstract

Objectives: Shwachman-Diamond syndrome is a rare disorder characterized by exocrine pancreatic insufficiency, skeletal abnormalities, and bone marrow failure, with high risk of leukemic evolution. The aim of the study was the immunophenotypic characterization of bone marrow cells from patients with Shwachman-Diamond syndrome to assess the maturation pathway of blood progenitor cells and to identify the presence of recurrent abnormalities. Methods: Bone marrow samples from nineteen patients and eleven controls were analyzed by multiparameter flow cytometry. Results: We found a low frequency of CD34+ cells (P=0.0179) and myeloid progenitors (P=0.025), in the bone marrow of patients with Shwachman-Diamond syndrome as compared to the controls. A significant reduction in the percentage of granulocytes (P=0.002) and an increase of monocytes (P

Original language	English
Pages (from-to)	308-315
Number of pages	8
Journal	European Journal of Haematology
Volume	95
Issue number	4
DOIs	https://doi.org/10.1111/ejh.12490
Publication status	Published - Oct 1 2015

Keywords

Flow cytometry
Immunophenotyping
Leukemia
Shwachman-Bodian-Diamond Syndrome (SBDS)
Stem cells

ASJC Scopus subject areas

Hematology

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10.1111/ejh.12490

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Immunophenotypic analysis of hematopoiesis in patients suffering from Shwachman-Bodian-Diamond Syndrome. / Mercuri, Angela; Cannata, Elisa; Perbellini, Omar; Cugno, Chiara; Balter, Rita; Zaccaron, Ada; Tridello, Gloria; Pizzolo, Giovanni; De Bortoli, Massimiliano; Krampera, Mauro; Cipolli, Marco; Cesaro, Simone.

In: European Journal of Haematology, Vol. 95, No. 4, 01.10.2015, p. 308-315.

Research output: Contribution to journal › Article › peer-review

Mercuri, Angela ; Cannata, Elisa ; Perbellini, Omar ; Cugno, Chiara ; Balter, Rita ; Zaccaron, Ada ; Tridello, Gloria ; Pizzolo, Giovanni ; De Bortoli, Massimiliano ; Krampera, Mauro ; Cipolli, Marco ; Cesaro, Simone. / Immunophenotypic analysis of hematopoiesis in patients suffering from Shwachman-Bodian-Diamond Syndrome. In: European Journal of Haematology. 2015 ; Vol. 95, No. 4. pp. 308-315.

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abstract = "Objectives: Shwachman-Diamond syndrome is a rare disorder characterized by exocrine pancreatic insufficiency, skeletal abnormalities, and bone marrow failure, with high risk of leukemic evolution. The aim of the study was the immunophenotypic characterization of bone marrow cells from patients with Shwachman-Diamond syndrome to assess the maturation pathway of blood progenitor cells and to identify the presence of recurrent abnormalities. Methods: Bone marrow samples from nineteen patients and eleven controls were analyzed by multiparameter flow cytometry. Results: We found a low frequency of CD34+ cells (P=0.0179) and myeloid progenitors (P=0.025), in the bone marrow of patients with Shwachman-Diamond syndrome as compared to the controls. A significant reduction in the percentage of granulocytes (P=0.002) and an increase of monocytes (P",

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author = "Angela Mercuri and Elisa Cannata and Omar Perbellini and Chiara Cugno and Rita Balter and Ada Zaccaron and Gloria Tridello and Giovanni Pizzolo and {De Bortoli}, Massimiliano and Mauro Krampera and Marco Cipolli and Simone Cesaro",

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AU - Balter, Rita

AU - Zaccaron, Ada

AU - Tridello, Gloria

AU - Pizzolo, Giovanni

AU - De Bortoli, Massimiliano

AU - Krampera, Mauro

AU - Cipolli, Marco

AU - Cesaro, Simone

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N2 - Objectives: Shwachman-Diamond syndrome is a rare disorder characterized by exocrine pancreatic insufficiency, skeletal abnormalities, and bone marrow failure, with high risk of leukemic evolution. The aim of the study was the immunophenotypic characterization of bone marrow cells from patients with Shwachman-Diamond syndrome to assess the maturation pathway of blood progenitor cells and to identify the presence of recurrent abnormalities. Methods: Bone marrow samples from nineteen patients and eleven controls were analyzed by multiparameter flow cytometry. Results: We found a low frequency of CD34+ cells (P=0.0179) and myeloid progenitors (P=0.025), in the bone marrow of patients with Shwachman-Diamond syndrome as compared to the controls. A significant reduction in the percentage of granulocytes (P=0.002) and an increase of monocytes (P

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Immunophenotypic analysis of hematopoiesis in patients suffering from Shwachman-Bodian-Diamond Syndrome

Abstract

Keywords

ASJC Scopus subject areas

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