Mast cells from different molecular and prognostic subtypes of systemic mastocytosis display distinct immunophenotypes
Mast cells from different molecular and prognostic subtypes of systemic mastocytosis display distinct immunophenotypes
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 itsclinical behavior
mastocytosis, immunophenotype, flow cytometry, KIT
mutations
719-726
Teodosio, Cristina
9d367222-6e90-4cc3-b466-f8b62523d4ec
García-Montero, Andrés C.
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Jara-Acevedo, María
bb3e3411-9357-4285-bf0a-9e5bd4c078f1
Sánchez-Muñoz, Laura
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Álvarez-Twose, Ivan
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Núñez, Rosa
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Schwartz, Lawrence B.
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Walls, Andrew F.
aaa7e455-0562-4b4c-94f5-ec29c74b1bfe
Escribano, Luis
af4d0195-fa49-4ed3-b8a8-49801fd51a0b
Orfao, Alberto
33ebfb71-8264-478b-b692-d0193b024aa7
March 2010
Teodosio, Cristina
9d367222-6e90-4cc3-b466-f8b62523d4ec
García-Montero, Andrés C.
8bee422a-ab36-4055-935c-a40971c971ee
Jara-Acevedo, María
bb3e3411-9357-4285-bf0a-9e5bd4c078f1
Sánchez-Muñoz, Laura
b4568e11-37af-483d-a47c-dde6d4997f16
Álvarez-Twose, Ivan
8edf5d5f-8931-4f54-8adf-897be7d71223
Núñez, Rosa
be101e94-3342-4418-96a9-5425ac87ad2d
Schwartz, Lawrence B.
85a3bfb8-0607-4229-8274-9d99de121135
Walls, Andrew F.
aaa7e455-0562-4b4c-94f5-ec29c74b1bfe
Escribano, Luis
af4d0195-fa49-4ed3-b8a8-49801fd51a0b
Orfao, Alberto
33ebfb71-8264-478b-b692-d0193b024aa7
Teodosio, Cristina, García-Montero, Andrés C., Jara-Acevedo, María, Sánchez-Muñoz, Laura, Álvarez-Twose, Ivan, Núñez, Rosa, Schwartz, Lawrence B., Walls, Andrew F., Escribano, Luis and Orfao, Alberto
(2010)
Mast cells from different molecular and prognostic subtypes of systemic mastocytosis display distinct immunophenotypes.
Journal of Allergy and Clinical Immunology, 125 (3), .
(doi:10.1016/j.jaci.2009.10.020).
(PMID:20061010)
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 itsclinical behavior
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Published date: March 2010
Keywords:
mastocytosis, immunophenotype, flow cytometry, KIT
mutations
Organisations:
Infection Inflammation & Immunity
Identifiers
Local EPrints ID: 166435
URI: http://eprints.soton.ac.uk/id/eprint/166435
ISSN: 0091-6749
PURE UUID: 048f4747-ae99-4f23-bb72-52347309fffa
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Date deposited: 29 Oct 2010 08:08
Last modified: 14 Mar 2024 02:34
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Contributors
Author:
Cristina Teodosio
Author:
Andrés C. García-Montero
Author:
María Jara-Acevedo
Author:
Laura Sánchez-Muñoz
Author:
Ivan Álvarez-Twose
Author:
Rosa Núñez
Author:
Lawrence B. Schwartz
Author:
Luis Escribano
Author:
Alberto Orfao
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