title: Blood CD34-c-Kit+ cell rate correlates with aggressive forms of systemic mastocytosis and behaves like a mast cell precursor.
abstract: Mastocytosis is a heterogeneous disease characterized by the accumulation of mast cells in one or more organs. Our objective was to identify a peripheral mast cell precursor and assess its variation rate in mastocytosis. A peripheral blood phenotypic analysis was performed among 50 patients with mastocytosis who were enrolled in a prospective multicentric French study, and the phenotypic analysis results of the patients were compared with those of healthy donors. The rate of peripheral blood CD34(-)c-Kit(+) cells correlated with the severity of mastocytosis. This cellular population was isolated from healthy donors as well as from patients with systemic mastocytosis. After 30 days of culture, the CD34(-)c-Kit(+) cells gave birth to mature mast cells, indicating that this cellular population constitutes a mast cell circulating precursor. Monitoring peripheral CD34(-)c-Kit(+) cells by flow cytometry could be a useful and low-invasive tool to determine the disease severity and the relapses and to assess treatment efficiency.
authors: Service d'Hématologie Adultes et Centre de Référence sur les Mastocytoses, Hôpital Necker-Enfants Malades, Université Paris Descartes, Assistance Publique-Hôpitaux de Paris, France. ohermine@gmail.com
authors: Sophie
authors: S
authors: Georgin-Lavialle
authors: Ludovic
authors: L
authors: Lhermitte
authors: Cédric
authors: C
authors: Baude
authors: Stéphane
authors: S
authors: Barete
authors: Julie
authors: J
authors: Bruneau
authors: Jean-Marie
authors: JM
authors: Launay
authors: Marie-Olivia
authors: MO
authors: Chandesris
authors: Katia
authors: K
authors: Hanssens
authors: Christian
authors: C
authors: de Gennes
authors: Gandhi
authors: G
authors: Damaj
authors: Fanny
authors: F
authors: Lanternier
authors: Mohamed
authors: M
authors: Hamidou
authors: Olivier
authors: O
authors: Lortholary
authors: Patrice
authors: P
authors: Dubreuil
authors: Frédéric
authors: F
authors: Feger
authors: Yves
authors: Y
authors: Lepelletier
authors: Olivier
authors: O
authors: Hermine
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018952:Antigens, CD34 / Q000097:blood*; D001773:Blood Cells / Q000378:metabolism* / Q000473:pathology*; D016022:Case-Control Studies; D002648:Child; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D008297:Male; D008407:Mast Cells / Q000378:metabolism / Q000473:pathology; D034721:Mastocytosis, Systemic / Q000097:blood* / Q000235:genetics; D008875:Middle Aged; D009154:Mutation; D011446:Prospective Studies; D019009:Proto-Oncogene Proteins c-kit / Q000097:blood* / Q000235:genetics; D013234:Stem Cells / Q000378:metabolism / Q000473:pathology
chemical_list: D018952:Antigens, CD34; D019009:Proto-Oncogene Proteins c-kit