Successful Imatinib Treatment for Systemic Mastocytosis Associated With Myelodysplastic/Myeloproliferative Neoplasm: Report of a Case and Literature Review

Systemic mastocytosis (SM) is a heterogeneous disease characterized by the expansion of mast cells in one or more tissues, frequently characterized by the presence of KITD816V mutation. The updated World Health Organization (WHO) classification of myeloid neoplasms recognizes SM with an associated h...

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Main Authors: Enrico Barozzi, Cristina Bucelli, Federica Irene Grifoni, Umberto Gianelli, Alessandra Iurlo, Daniele Cattaneo
Format: Article
Language:English
Published: Frontiers Media S.A. 2022-01-01
Series:Frontiers in Oncology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fonc.2021.819097/full
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author Enrico Barozzi
Enrico Barozzi
Cristina Bucelli
Federica Irene Grifoni
Umberto Gianelli
Umberto Gianelli
Alessandra Iurlo
Daniele Cattaneo
Daniele Cattaneo
author_facet Enrico Barozzi
Enrico Barozzi
Cristina Bucelli
Federica Irene Grifoni
Umberto Gianelli
Umberto Gianelli
Alessandra Iurlo
Daniele Cattaneo
Daniele Cattaneo
author_sort Enrico Barozzi
collection DOAJ
description Systemic mastocytosis (SM) is a heterogeneous disease characterized by the expansion of mast cells in one or more tissues, frequently characterized by the presence of KITD816V mutation. The updated World Health Organization (WHO) classification of myeloid neoplasms recognizes SM with an associated hematological neoplasm (SM-AHN) as a new subtype among the others, which is depicted by the coexistence of SM with another hematological clonal disease. Prognosis is very different among SM patients, while its treatment, although highly personalized, is still challenging. Here we report a case of KITD816V-unmutated SM associated with MDS/MPN successfully treated with imatinib.
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spelling doaj.art-fc82bd787821420485aa7157fd39a4502022-12-21T16:35:02ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2022-01-011110.3389/fonc.2021.819097819097Successful Imatinib Treatment for Systemic Mastocytosis Associated With Myelodysplastic/Myeloproliferative Neoplasm: Report of a Case and Literature ReviewEnrico Barozzi0Enrico Barozzi1Cristina Bucelli2Federica Irene Grifoni3Umberto Gianelli4Umberto Gianelli5Alessandra Iurlo6Daniele Cattaneo7Daniele Cattaneo8Hematology Division, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyDepartment of Oncology and Hemato-Oncology, University of Milan, Milan, ItalyHematology Division, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyHematology Division, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyDepartment of Pathophysiology and Transplantation, University of Milan, Milan, ItalyDivision of Pathology, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyHematology Division, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyHematology Division, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyDepartment of Oncology and Hemato-Oncology, University of Milan, Milan, ItalySystemic mastocytosis (SM) is a heterogeneous disease characterized by the expansion of mast cells in one or more tissues, frequently characterized by the presence of KITD816V mutation. The updated World Health Organization (WHO) classification of myeloid neoplasms recognizes SM with an associated hematological neoplasm (SM-AHN) as a new subtype among the others, which is depicted by the coexistence of SM with another hematological clonal disease. Prognosis is very different among SM patients, while its treatment, although highly personalized, is still challenging. Here we report a case of KITD816V-unmutated SM associated with MDS/MPN successfully treated with imatinib.https://www.frontiersin.org/articles/10.3389/fonc.2021.819097/fullimatinibsystemic mastocytosisSM-AHNMDS/MPNmyeloid neoplasms
spellingShingle Enrico Barozzi
Enrico Barozzi
Cristina Bucelli
Federica Irene Grifoni
Umberto Gianelli
Umberto Gianelli
Alessandra Iurlo
Daniele Cattaneo
Daniele Cattaneo
Successful Imatinib Treatment for Systemic Mastocytosis Associated With Myelodysplastic/Myeloproliferative Neoplasm: Report of a Case and Literature Review
Frontiers in Oncology
imatinib
systemic mastocytosis
SM-AHN
MDS/MPN
myeloid neoplasms
title Successful Imatinib Treatment for Systemic Mastocytosis Associated With Myelodysplastic/Myeloproliferative Neoplasm: Report of a Case and Literature Review
title_full Successful Imatinib Treatment for Systemic Mastocytosis Associated With Myelodysplastic/Myeloproliferative Neoplasm: Report of a Case and Literature Review
title_fullStr Successful Imatinib Treatment for Systemic Mastocytosis Associated With Myelodysplastic/Myeloproliferative Neoplasm: Report of a Case and Literature Review
title_full_unstemmed Successful Imatinib Treatment for Systemic Mastocytosis Associated With Myelodysplastic/Myeloproliferative Neoplasm: Report of a Case and Literature Review
title_short Successful Imatinib Treatment for Systemic Mastocytosis Associated With Myelodysplastic/Myeloproliferative Neoplasm: Report of a Case and Literature Review
title_sort successful imatinib treatment for systemic mastocytosis associated with myelodysplastic myeloproliferative neoplasm report of a case and literature review
topic imatinib
systemic mastocytosis
SM-AHN
MDS/MPN
myeloid neoplasms
url https://www.frontiersin.org/articles/10.3389/fonc.2021.819097/full
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