Analysis of Gene Mutation and Clinical Characteristics in 19 Children with Juvenile Myelomonocytic Leukemia.
10.19746/j.cnki.issn.1009-2137.2020.06.004
- Author:
Kai-Zhi WENG
1
;
Yong-Zhi ZHENG
2
;
Shu-Quan ZHUANG
3
;
Hai-Yun CHEN
4
;
Shao-Hua LE
5
Author Information
1. Department of Pediatrics, Zhangzhou Affiliated Hospital of Fujian Medical University, Zhangzhou 363000, Fujian Province, China.
2. Department of Pediatric Hematology, Fujian Institute of Hematology, Fujian Provincial Key Laboratory of Hematology, Fujian Blood Medical Center, Fujian Medical University Union Hospital, Fuzhou 350001, Fujian Province, China.
3. Department of Pediatrics, Quanzhou First Hospital Affiliated to Fujian Medical University, Quanzhou 362000, Fujian Province, China.
4. Department of Pediatrics, Longyan First Hospital, Longyan 364000, Fujian Province, China.
5. Department of Pediatric Hematology, Fujian Institute of Hematology, Fujian Provincial Key Laboratory of Hematology, Fujian Blood Medical Center, Fujian Medical University Union Hospital, Fuzhou 350001, Fujian Province, China,E-mail: lele883@sina.com.
- Publication Type:Journal Article
- MeSH:
Child;
Female;
Hematopoietic Stem Cell Transplantation;
Humans;
Infant;
Leukemia, Myelomonocytic, Juvenile/genetics*;
Male;
Mutation;
Prognosis;
Protein Tyrosine Phosphatase, Non-Receptor Type 11/genetics*;
Retrospective Studies
- From:
Journal of Experimental Hematology
2020;28(6):1811-1818
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVE:To analyze the gene mutations of children with juvenile myelomonocytic leukemia (JMML) and their correlation with clinical characteristics.
METHODS:The genetic mutation results and clinical data of 19 children with JMML in Fujian from January 2015 to December 2018 were collected and analyzed retrospectively. According to the results of gene mutation, they were divided into PTPN11 gene mutation group and non-PTPN11 gene mutation group, and the clinical characteristics and prognosis of children with JMML between two groups were compared.
RESULTS:Among the 19 children with JMML, 14 cases were male and 5 cases were female, and male/female ratio was 2.8∶1. The median age at diagnosis was 13(3-48) months, and 14 cases (73.68%) were less than 2 years old. Abdominal distension and pyrexia were the common initial symptoms, and all the children with JMML had splenomegaly. The median white blood cell count was 39.82(4.53-103.4)×10
CONCLUSION:JMML is more common in male infancy and toddlerhood, and the main gene mutation types are PTPN11 and Ras mutations. Because the JMML children with PTPN11 mutations show particularly rapid disease progression, if there is no timely intervention, most children die in a short period of time. Therefore, early HSCT may improve the prognosis of the children with JMML.
