Comprehensive mastocytosis data analysis from a single center

BMC Cancer

Research

Can Baykal¹  Aslı Gelincik²  Simge Erdem³  Tarık Onur Tiryaki³  Akif Selim Yavuz³  Sıdıka Gülkan Özkan³  İpek Yönal Hindilerden³  Meliha Nalçacı³  Aynur Dağlar Aday⁴  Nesimi Büyükbabani⁵  Gülçin Yegen⁵  İbrahim Öner Doğan⁵ 

[1] Faculty of Medicine, Department of Dermatology, Istanbul University, Istanbul, Turkey;Faculty of Medicine, Department of Internal Medicine, Division of Allergy, Istanbul University, Istanbul, Turkey;Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Istanbul University, Istanbul, Turkey;Faculty of Medicine, Department of Internal Medicine, Division of Medical Genetics, Istanbul University, Istanbul, Turkey;Faculty of Medicine, Department of Pathology, Istanbul University, Istanbul, Turkey;

关键词: Mastocytosis;    An orphan disease;    Comprehensive analysis;    Cytoreductive therapy;    Extended clinical Spectrum;   

DOI  :  10.1186/s12885-022-10498-3

 received in 2022-06-27, accepted in 2022-12-30,  发布年份 2022

来源: Springer

【 摘 要 】

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.

【 授权许可】

CC BY   
© The Author(s) 2023