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. 2023 Jan 24;23(1):82.
doi: 10.1186/s12885-022-10498-3.

Comprehensive mastocytosis data analysis from a single center

Tarık Onur Tiryaki  1 , Sıdıka Gülkan Özkan  2 , Simge Erdem  2 , Aynur Dağlar Aday  3 , İpek Yönal Hindilerden  2 , Aslı Gelincik  4 , Can Baykal  5 , Gülçin Yegen  6 , İbrahim Öner Doğan  6 , Nesimi Büyükbabani  6 , Meliha Nalçacı  2 , Akif Selim Yavuz  2
Affiliations

Comprehensive mastocytosis data analysis from a single center

Tarık Onur Tiryaki et al. BMC Cancer. .

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.

Keywords: An orphan disease; Comprehensive analysis; Cytoreductive therapy; Extended clinical Spectrum; Mastocytosis.

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Conflict of interest statement

The authors declare that they have no conflict of interest.

Figures

Fig. 1
Fig. 1
A Survival chart by subgroups with WHO classification. B Survival chart according to the IPSM score
See this image and copyright information in PMC

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