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Open Access 01-12-2023 | Mastocytosis | Research

Comprehensive mastocytosis data analysis from a single center

Authors: Tarık Onur Tiryaki, Sıdıka Gülkan Özkan, Simge Erdem, Aynur Dağlar Aday, İpek Yönal Hindilerden, Aslı Gelincik, Can Baykal, Gülçin Yegen, İbrahim Öner Doğan, Nesimi Büyükbabani, Meliha Nalçacı, Akif Selim Yavuz

Published in: BMC Cancer | Issue 1/2023

Abstract

Mastocytosis is a very rare disorder and is divided into three prognostically distinct variants by World Health Organization: Cutaneous mastocytosis (CM), systemic mastocytosis (SM), and mast cell sarcoma or localized mast cell (MC) tumors. The wide range of complaints may cause patients to consult various clinics, with resulting mis- or underdiagnosis. Therefore, cooperation between different subspecialties is of paramount importance. In this article, we have compiled 104 adult mastocytosis cases diagnosed and followed in our Hematology and other clinics. 86 (82.7%) of 104 patients had systemic mastocytosis. Osteoporosis, disease-related complications, and secondary malignancies are important topics in this group. We know that indolent form has great survival. But smoldering or aggressive mastocytosis has a poor prognosis. CM and indolent SM have a significantly better prognosis compared to aggressive SM (p < 0.001). We found that the presence of more than 25% of mast cells in the bone marrow, the presence of concomitant marrow dysplasia, and the presence of disease-related complications affect survival (p < 0.001). In addition to the WHO classification, the IPSM scoring system is indicative of the prognosis in this rare disease.

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Title: Comprehensive mastocytosis data analysis from a single center
Authors: Tarık Onur Tiryaki
Sıdıka Gülkan Özkan
Simge Erdem
Aynur Dağlar Aday
İpek Yönal Hindilerden
Aslı Gelincik
Can Baykal
Gülçin Yegen
İbrahim Öner Doğan
Nesimi Büyükbabani
Meliha Nalçacı
Akif Selim Yavuz
Publication date: 01-12-2023
Publisher: BioMed Central
Keywords: Mastocytosis
Cutaneous Mastocytosis
Systemic Mastocytosis
Orphan Diseases
Published in: BMC Cancer / Issue 1/2023
Electronic ISSN: 1471-2407
DOI: https://doi.org/10.1186/s12885-022-10498-3

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