JUVENILE MYELOMONOCYTIC LEUKEMIA SINGLE CENTER EXPERIENCE
Objective: In the 5-year follow-up of patients diagnosed with Juvenile Myelomonocytic Leukemia (JMML) in a single center; reveal treatment and survival analyzes Methodology: In this study, clinical and laboratory data of 12 JMML patients followed in Ankara Pediatric Hematology Hospital and Ankara Ci...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2021-11-01
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| Series: | Hematology, Transfusion and Cell Therapy |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2531137921011408 |
| _version_ | 1818754420541751296 |
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| author | Yunus Murat Akcabelen Dilek Kaçar Ayça Koca Yozgat Özlem Arman Bilir Dilek Gürlek Gökçebay Turan Bayhan İkbal Ok Bozkaya Namık Yaşar Özbek Neşe Yaralı |
| author_facet | Yunus Murat Akcabelen Dilek Kaçar Ayça Koca Yozgat Özlem Arman Bilir Dilek Gürlek Gökçebay Turan Bayhan İkbal Ok Bozkaya Namık Yaşar Özbek Neşe Yaralı |
| author_sort | Yunus Murat Akcabelen |
| collection | DOAJ |
| description | Objective: In the 5-year follow-up of patients diagnosed with Juvenile Myelomonocytic Leukemia (JMML) in a single center; reveal treatment and survival analyzes Methodology: In this study, clinical and laboratory data of 12 JMML patients followed in Ankara Pediatric Hematology Hospital and Ankara City Hospital Pediatric Hematology Clinics between 2015-2020 were analyzed retrospectively. Results: The median age at diagnosis was 1.7 years (0.23-5.7). Monosomy 7, 4 PTPN11, 2 NRAS, 4 KRAS, 1 CBL mutations were detected in 2 of the patients. Hematopoietic stem cell transplantation was performed in 8 of the patients. Before transplantation, 7 patients had received a median of 4 cycles of azacitidine treatment. The mean time from diagnosis to transplantation was 15 months (1-29 months). The 5-year overall survival at median 15-month follow-up was 50%. Conclusion: With hypomethylating agents and HSCT, survival in JMML improves compared to historical control groups. However, further multicenter prospective studies are needed to prevent long-term mortality and morbidity. |
| first_indexed | 2024-12-18T05:22:58Z |
| format | Article |
| id | doaj.art-a72aeab0c8db4fee96fe6f0fc022886b |
| institution | Directory Open Access Journal |
| issn | 2531-1379 |
| language | English |
| last_indexed | 2024-12-18T05:22:58Z |
| publishDate | 2021-11-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Hematology, Transfusion and Cell Therapy |
| spelling | doaj.art-a72aeab0c8db4fee96fe6f0fc022886b2022-12-21T21:19:36ZengElsevierHematology, Transfusion and Cell Therapy2531-13792021-11-0143S25JUVENILE MYELOMONOCYTIC LEUKEMIA SINGLE CENTER EXPERIENCEYunus Murat Akcabelen0Dilek Kaçar1Ayça Koca Yozgat2Özlem Arman Bilir3Dilek Gürlek Gökçebay4Turan Bayhan5İkbal Ok Bozkaya6Namık Yaşar Özbek7Neşe Yaralı8University of Health Sciences, Ankara City Hospital, Pediatric Hematology and OncologyUniversity of Health Sciences, Ankara City Hospital, Pediatric Hematology and OncologyUniversity of Health Sciences, Ankara City Hospital, Pediatric Hematology and OncologyUniversity of Health Sciences, Ankara City Hospital, Pediatric Hematology and OncologyUniversity of Health Sciences, Ankara City Hospital, Pediatric Hematology and OncologyUniversity of Health Sciences, Ankara City Hospital, Pediatric Hematology and OncologyUniversity of Health Sciences, Ankara City Hospital, Pediatric Hematology and OncologyUniversity of Health Sciences, Ankara City Hospital, Pediatric Hematology and OncologyYildirim Beyazit University, Ankara City Hospital, Pediatric GastroenterologyObjective: In the 5-year follow-up of patients diagnosed with Juvenile Myelomonocytic Leukemia (JMML) in a single center; reveal treatment and survival analyzes Methodology: In this study, clinical and laboratory data of 12 JMML patients followed in Ankara Pediatric Hematology Hospital and Ankara City Hospital Pediatric Hematology Clinics between 2015-2020 were analyzed retrospectively. Results: The median age at diagnosis was 1.7 years (0.23-5.7). Monosomy 7, 4 PTPN11, 2 NRAS, 4 KRAS, 1 CBL mutations were detected in 2 of the patients. Hematopoietic stem cell transplantation was performed in 8 of the patients. Before transplantation, 7 patients had received a median of 4 cycles of azacitidine treatment. The mean time from diagnosis to transplantation was 15 months (1-29 months). The 5-year overall survival at median 15-month follow-up was 50%. Conclusion: With hypomethylating agents and HSCT, survival in JMML improves compared to historical control groups. However, further multicenter prospective studies are needed to prevent long-term mortality and morbidity.http://www.sciencedirect.com/science/article/pii/S2531137921011408 |
| spellingShingle | Yunus Murat Akcabelen Dilek Kaçar Ayça Koca Yozgat Özlem Arman Bilir Dilek Gürlek Gökçebay Turan Bayhan İkbal Ok Bozkaya Namık Yaşar Özbek Neşe Yaralı JUVENILE MYELOMONOCYTIC LEUKEMIA SINGLE CENTER EXPERIENCE Hematology, Transfusion and Cell Therapy |
| title | JUVENILE MYELOMONOCYTIC LEUKEMIA SINGLE CENTER EXPERIENCE |
| title_full | JUVENILE MYELOMONOCYTIC LEUKEMIA SINGLE CENTER EXPERIENCE |
| title_fullStr | JUVENILE MYELOMONOCYTIC LEUKEMIA SINGLE CENTER EXPERIENCE |
| title_full_unstemmed | JUVENILE MYELOMONOCYTIC LEUKEMIA SINGLE CENTER EXPERIENCE |
| title_short | JUVENILE MYELOMONOCYTIC LEUKEMIA SINGLE CENTER EXPERIENCE |
| title_sort | juvenile myelomonocytic leukemia single center experience |
| url | http://www.sciencedirect.com/science/article/pii/S2531137921011408 |
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