Mast cells from different molecular and prognostic subtypes of systemic mastocytosis display distinct immunophenotypes - 07/08/11
, Andrés C. García-Montero, PhD a, , María Jara-Acevedo, MSc a, Laura Sánchez-Muñoz, MD, PhD b, Ivan Álvarez-Twose, MD b, Rosa Núñez, MD c, Lawrence B. Schwartz, MD, PhD d, Andrew F. Walls, PhD e, Luis Escribano, MD, PhD b, , Alberto Orfao, MD, PhD a, ⁎, 
Reprint requests: Alberto Orfao, MD, PhD, Centro de Investigación del Cáncer, Avenida Universidad de Coimbra s/n, Campus Miguel de Unamuno, 37007, Salamanca, Spain.Abstract |
Background |
Systemic mastocytosis (SM) is a heterogeneous group of disorders with distinct clinical and biological behavior. Despite this, little is known about the immunophenotypic features of the distinct diagnostic categories of SM.
Objective |
To analyze the immunophenotypic characteristics of bone marrow (BM) mast cells (MCs) of different subtypes of SM.
Methods |
Bone marrow samples from 123 patients with different subtypes of SM and 92 controls were analyzed for a broad panel of immunophenotypic markers by flow cytometry.
Results |
Three clearly different maturation-associated immunophenotypic profiles were found for BMMCs in SM. These different profiles were associated with both genetic markers of the disease and its clinical behavior. BMMCs from poor-prognosis categories of SM (aggressive SM and MC leukemia) typically showed an immature phenotype with clonal involvement of all myeloid lineages by the D816V stem cell growth factor receptor gene (KIT) mutation. In turn, a mature activated versus resting BMMC immunophenotype was commonly found among patients with good-prognosis subtypes of SM depending on whether they carried (indolent SM and clonal MC activation disorders) or not (well differentiated SM) the D816V KIT mutation.
Conclusion |
Bone marrow MCs from SM show 3 different maturation-related immunophenotypic profiles that are associated with both the genetic markers of the disease and its clinical behavior.
Le texte complet de cet article est disponible en PDF.Key words : Mastocytosis, immunophenotype, flow cytometry, KIT mutations
Abbreviations used : ASM, ASM-AHNMD, BM, cMCAD, CPA, CyB12, CyG5, FDR, ISM, ISM-AHNMD, MC, MCL, NPV, PPV, SM, SM-AHNMD, SSC, sT, TN, TP, WDSM
Plan
| Supported by grants from the Fondo de Investigaciones Sanitarias of the Ministerio de Sanidad y Consumo of Spain (REMA G03/007, PI050726, PI061377, PI060529, and RETICS RD06/0020/0035-FEDER); Junta de Castilla y León (Grant SAN/1778/2009); Junta de Comunidades de Castilla La Mancha (FISCAM 2007/36), and Fundación MMA. A.C.G.-M. is supported by a grant from Fondo de Investigaciones Sanitarias/FEDER (CP03/00035). C.T. is supported by a grant from the Fundação para a Ciência e Tecnologia of Portugal (SFRH/BD/17545/2004). L.B.S. is supported by grants from the National Institutes of Health (AI27517 and AI077435). |
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| Disclosure of potential conflict of interest: L. B. Schwartz is on the speakers’ bureau and is a consultant for Novartis/Genentech; is the inventor of the tryptase assay for Phadia; receives grant support from the NIH, GlaxoSmithKline, Novartis/Genentech, Pharming, and Ception; has provided legal consultation or expert witness testimony in cases related to anaphylaxis; is on the Board of Directors for AAFA and CIS; and is on the Program Directors’ Board for the AAAAI. The rest of the authors have declared that they have no conflict of interest. |
Vol 125 - N° 3
P. 719 - mars 2010 Retour au numéro
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