AI Article Synopsis
- Systemic mastocytosis is a rare disease that varies in severity, with most patients experiencing mild symptoms, but some may develop aggressive forms.
- Complications can arise from an increase in mast cells or the presence of other blood disorders, leading to worse outcomes for those affected.
- Recent advancements in diagnosis and treatment have improved prognosis for patients with advanced systemic mastocytosis, addressing key questions about classification and management.
Article Abstract
Systemic mastocytosis is a rare disease which is being better recognized and managed. While the vast majority of patients have indolent disease with variable symptom burden, a small proportion evolve or present with aggressive disease. This may be due to increases in mast cell burden (leukemic, associated with tumour masses) or more commonly due to the presence of an additional hematologic neoplasm (SM-AHN). These patients with advanced systemic mastocytosis have poor outcome; however, recent advances in diagnosis, molecular genetics and treatment have changed the prognostic landscape for this group of patients. In this review we address the most topical questions related to diagnostics, classification, new disease entities, treatment and multiparameter prognostic scoring systems.
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| http://dx.doi.org/10.1016/j.beha.2022.101380 | DOI Listing |
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