Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification.

ABSTRACT:

Background

In indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM.

Methods

We employed a dataset of 1993 patients from the registry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM.

Results

We found that overall survival (OS) is worse in ISM compared to CM. Moreover, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of disease progressions have been observed (4.9%

SUBMITTER: Trizuljak J 

PROVIDER: S-EPMC7115854 | biostudies-literature | 2020 Aug

REPOSITORIES: biostudies-literature