The University of Southampton
University of Southampton Institutional Repository

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
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
0091-6749
719-726
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
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), 719-726. (doi:10.1016/j.jaci.2009.10.020). (PMID:20061010)

Record type: Article

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

This record has no associated files available for download.

More information

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
ORCID for Andrew F. Walls: ORCID iD orcid.org/0000-0003-4803-4595

Catalogue record

Date deposited: 29 Oct 2010 08:08
Last modified: 14 Mar 2024 02:34

Export record

Altmetrics

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: Andrew F. Walls ORCID iD
Author: Luis Escribano
Author: Alberto Orfao

Download statistics

Downloads from ePrints over the past year. Other digital versions may also be available to download e.g. from the publisher's website.

View more statistics

Atom RSS 1.0 RSS 2.0

Contact ePrints Soton: eprints@soton.ac.uk

ePrints Soton supports OAI 2.0 with a base URL of http://eprints.soton.ac.uk/cgi/oai2

This repository has been built using EPrints software, developed at the University of Southampton, but available to everyone to use.

We use cookies to ensure that we give you the best experience on our website. If you continue without changing your settings, we will assume that you are happy to receive cookies on the University of Southampton website.

×