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...

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Autores principales: Yunus Murat Akcabelen, Dilek Kaçar, Ayça Koca Yozgat, Özlem Arman Bilir, Dilek Gürlek Gökçebay, Turan Bayhan, İkbal Ok Bozkaya, Namık Yaşar Özbek, Neşe Yaralı
Formato: article
Lenguaje:EN
Publicado: Elsevier 2021
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Acceso en línea:https://doaj.org/article/a72aeab0c8db4fee96fe6f0fc022886b
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Sumario: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.