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Article

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

European journal of haematology - 1 Oct 2015

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

Abstract excerpt

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...

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PMID: 25402872
DOI: 10.1111/ejh.12490

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