Abstract
ABSTRACTObjective: To investigate clinical features, diagnosis, treatment strategies and prognosis of juvenile myelomonocytic leukemia (JMML).Methods: The clinical data of 21 patients with JMML who were diagnosed in our hospital from January 2013 to May 2018 were retrospectively analyzed.Results: Among the 21 children with JMML, 16 were male and 5 were female. Out of the 21 children who were diagnosed with JMML, 7 were lost after treatment while the remaining 14 received A-3V chemotherapy regimen of South Korea. The effective response rate was 78.5%. The three-year overall survival (OS) rate and three-year disease-free survival (DFS) rate were (76.2 ± 14.8)% and (66.2 ± 14)%, respectively. Single factor analysis showed that PLT count ≤33×109/L, LDH level >500 U/L and HbF level >10% and chemotherapy only were the significant factors that lead to poor prognosis in children. Cox multivariate analysis showed that the choice of treatment options affected the prognosis of JMML children. By taking prognostic factors for long-term efficacy into account, patients with treatment strategy of chemotherapy alongside hematopoietic stem cell transplantation (HSCT) have a better prognosis.Conclusion: The PLT count, LDH level, HbF level and choice of treatment plan are important for the evaluation of prognosis for children with JMML. Although there is a lack of consistency in terms of donors but the A-3V scheme is relatively stable, so HSCT should be preferred for children with poor prognostic factors.
Highlights
Treatment response and its influencing factors Out of the 21 children who were diagnosed with juvenile myelomonocytic leukemia (JMML), 7 were lost after treatment while the remaining 14 received chemotherapy from A-3 V in South Korea
JMML is a kind of invasive myeloid disease in children
Of the 17 patients who were transferred to our hospital due to unclear diagnosis, 4 patients were misdiagnosed as other diseases in the other hospitals
Summary
There were a total of 21 patients with JMML, including 16 males and 5 females. Treatment regimen involves chemotherapy and induced differentiation therapy that were performed. Efficacy evaluation and follow-up procedures are based on the consensus by the international JMML collaboration group in 2015 [4]. The parameters of early treatment response evaluation include 6 clinical features and 3 genetic characteristics. It is divided into three levels according to the efficacy: complete remission, partial remission and disease progression. Clinical characteristics were analyzed and compared using descriptive analysis, corrected chisquare test and exact probability method; survival analysis was done by using Kaplan–Meier survival curve whereas single factor analysis was done through the Log-rank test.
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