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Comprehensive mastocytosis data analysis from a single center.
Tarık Onur Tiryaki1, Sıdıka Gülkan Özkan2, Simge Erdem2
1Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Istanbul University, Istanbul, Turkey. tonurtiryaki@gmail.com.
BMC Cancer
|January 24, 2023
Summary
Mastocytosis, a rare disorder, includes cutaneous and systemic forms. Aggressive systemic mastocytosis has a poor prognosis, unlike indolent forms, with key survival factors identified.
Area of Science:
- Hematology
- Oncology
- Rare Diseases
Background:
- Mastocytosis is a rare disorder with distinct variants: Cutaneous Mastocytosis (CM), Systemic Mastocytosis (SM), and mast cell tumors.
- Diverse symptoms lead to diagnostic challenges, highlighting the need for interdisciplinary collaboration.
Purpose of the Study:
- To analyze prognostic factors and survival in adult mastocytosis cases.
- To evaluate the utility of the WHO classification and the IPSM scoring system.
Main Methods:
- Retrospective analysis of 104 adult mastocytosis cases diagnosed and followed in Hematology and other clinics.
- Review of patient data including bone marrow mast cell percentage, dysplasia, complications, and survival outcomes.
Main Results:
- Systemic Mastocytosis (SM) was diagnosed in 86% of patients.
- Aggressive SM showed a significantly poorer prognosis than indolent SM (p < 0.001).
- Bone marrow mast cell infiltration (>25%), marrow dysplasia, and disease complications were critical survival predictors (p < 0.001).
Conclusions:
- The WHO classification effectively distinguishes prognostic groups within mastocytosis.
- The IPSM scoring system provides valuable prognostic information for rare mast cell disorders.
- Early identification of prognostic factors is crucial for managing mastocytosis patients.
