Comprehensive mastocytosis data analysis from a single center
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...
| Main Authors: | , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2023-01-01
|
| Series: | BMC Cancer |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12885-022-10498-3 |
| _version_ | 1828051673627492352 |
|---|---|
| author | 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 |
| author_facet | 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 |
| author_sort | Tarık Onur Tiryaki |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-10T19:42:18Z |
| format | Article |
| id | doaj.art-b3e0e63abb90432b9167a0783d6e5362 |
| institution | Directory Open Access Journal |
| issn | 1471-2407 |
| language | English |
| last_indexed | 2024-04-10T19:42:18Z |
| publishDate | 2023-01-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Cancer |
| spelling | doaj.art-b3e0e63abb90432b9167a0783d6e53622023-01-29T12:14:44ZengBMCBMC Cancer1471-24072023-01-0123111110.1186/s12885-022-10498-3Comprehensive mastocytosis data analysis from a single centerTarık Onur Tiryaki0Sıdıka Gülkan Özkan1Simge Erdem2Aynur Dağlar Aday3İpek Yönal Hindilerden4Aslı Gelincik5Can Baykal6Gülçin Yegen7İbrahim Öner Doğan8Nesimi Büyükbabani9Meliha Nalçacı10Akif Selim Yavuz11Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Istanbul UniversityFaculty of Medicine, Department of Internal Medicine, Division of Hematology, Istanbul UniversityFaculty of Medicine, Department of Internal Medicine, Division of Hematology, Istanbul UniversityFaculty of Medicine, Department of Internal Medicine, Division of Medical Genetics, Istanbul UniversityFaculty of Medicine, Department of Internal Medicine, Division of Hematology, Istanbul UniversityFaculty of Medicine, Department of Internal Medicine, Division of Allergy, Istanbul UniversityFaculty of Medicine, Department of Dermatology, Istanbul UniversityFaculty of Medicine, Department of Pathology, Istanbul UniversityFaculty of Medicine, Department of Pathology, Istanbul UniversityFaculty of Medicine, Department of Pathology, Istanbul UniversityFaculty of Medicine, Department of Internal Medicine, Division of Hematology, Istanbul UniversityFaculty of Medicine, Department of Internal Medicine, Division of Hematology, Istanbul UniversityAbstract 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.https://doi.org/10.1186/s12885-022-10498-3MastocytosisAn orphan diseaseComprehensive analysisCytoreductive therapyExtended clinical Spectrum |
| spellingShingle | 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 Comprehensive mastocytosis data analysis from a single center BMC Cancer Mastocytosis An orphan disease Comprehensive analysis Cytoreductive therapy Extended clinical Spectrum |
| title | Comprehensive mastocytosis data analysis from a single center |
| title_full | Comprehensive mastocytosis data analysis from a single center |
| title_fullStr | Comprehensive mastocytosis data analysis from a single center |
| title_full_unstemmed | Comprehensive mastocytosis data analysis from a single center |
| title_short | Comprehensive mastocytosis data analysis from a single center |
| title_sort | comprehensive mastocytosis data analysis from a single center |
| topic | Mastocytosis An orphan disease Comprehensive analysis Cytoreductive therapy Extended clinical Spectrum |
| url | https://doi.org/10.1186/s12885-022-10498-3 |
| work_keys_str_mv | AT tarıkonurtiryaki comprehensivemastocytosisdataanalysisfromasinglecenter AT sıdıkagulkanozkan comprehensivemastocytosisdataanalysisfromasinglecenter AT simgeerdem comprehensivemastocytosisdataanalysisfromasinglecenter AT aynurdaglaraday comprehensivemastocytosisdataanalysisfromasinglecenter AT ipekyonalhindilerden comprehensivemastocytosisdataanalysisfromasinglecenter AT aslıgelincik comprehensivemastocytosisdataanalysisfromasinglecenter AT canbaykal comprehensivemastocytosisdataanalysisfromasinglecenter AT gulcinyegen comprehensivemastocytosisdataanalysisfromasinglecenter AT ibrahimonerdogan comprehensivemastocytosisdataanalysisfromasinglecenter AT nesimibuyukbabani comprehensivemastocytosisdataanalysisfromasinglecenter AT melihanalcacı comprehensivemastocytosisdataanalysisfromasinglecenter AT akifselimyavuz comprehensivemastocytosisdataanalysisfromasinglecenter |