Clinical analysis of seven cases of juvenile myelomonocytic leukemia / 中国基层医药

ABSTRACT

Objectives:To analyze the clinical features of juvenile myelomonocytic leukemia (JMML) and investigate the characteristics of diagnosis and treatment of this disease.Methods:The clinical data of seven children patients with JMML who received treatment in The First Affiliated Hospital of Xinxiang Medical University between April 2015 and February 2020 were retrospectively analyzed. The clinical efficacy of different treatments was analyzed.Results:The median age at diagnosis of JMML was 8 months and 4 days for seven children patients. Fever was the principal cause of treatment, and it was mostly accompanied by hepatosplenomegaly. The median value of peripheral blood leukocyte count was 36.1 × 10 9/L, and it was 4.5 × 10 9/L for mononuclear cell count, 88 g/L for hemoglobin level, and 47 × 10 9/L for platelet count. Myeloid immature cells were found in peripheral blood smears of six patients. Chromosome examination results revealed 7-monomer in one patient, and normal karyotype in six patients. Hemoglobin level was increased in six patients. Gene detection results revealed PTPN11+NF1 mutation in one patient, N-RAS mutation in two patients, and K-RAS mutation in one patient. Three patients gave up treatment, three patients received low-intensity chemotherapy , and these six patients died of complicated infection. One patient received allogeneic hematopoietic stem cell transplantation and the patient survived without any event after 14 months of follow-up. Conclusion:The age of JMML onset is low. JMML has poor clinical specificity. Gene detection is helpful for early diagnosis of JMML. Low-intensity chemotherapy can prolong survival period, but it can not improve prognosis. Infection is the principal cause of death in patients with JMML. Hematopoietic stem cell transplantation is the only possible method to cure the disease.