AUTHOR=Barozzi Enrico , Bucelli Cristina , Grifoni Federica Irene , Gianelli Umberto , Iurlo Alessandra , Cattaneo Daniele 

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

JOURNAL=Frontiers in Oncology

VOLUME=11

YEAR=2022

URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2021.819097

DOI=10.3389/fonc.2021.819097

ISSN=2234-943X

ABSTRACT=<p>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 <italic>KIT</italic>D816V 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 <italic>KIT</italic>D816V-unmutated SM associated with MDS/MPN successfully treated with imatinib.</p>