Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jan 6, 2021; 9(1): 204-210
Published online Jan 6, 2021. doi: 10.12998/wjcc.v9.i1.204
Myeloid neoplasm with eosinophilia and rearrangement of platelet-derived growth factor receptor beta gene in children: Two case reports
Shi-Chong Wang, Wen-Yu Yang
Shi-Chong Wang, Wen-Yu Yang, State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China
Author contributions: Wang SC collected the data and wrote the manuscript; Yang WY revised the manuscript for important intellectual content and English language; All authors approved the final version of the manuscript.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: The authors declare no conflicts of interest.
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Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Wen-Yu Yang, PhD, Chief Doctor, State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 288 Nanjing Road, Tianjin 300020, China. yangwenyu@ihcams.ac.cn
Received: June 24, 2020
Peer-review started: June 24, 2020
First decision: October 18, 2020
Revised: October 22, 2020
Accepted: November 12, 2020
Article in press: November 12, 2020
Published online: January 6, 2021
Processing time: 190 Days and 21.9 Hours
Abstract
BACKGROUND

Myeloid neoplasm (MN) with eosinophilia and rearrangement of platelet-derived growth factor receptor beta (PDGFRB) shows a good therapeutic response to imatinib in adults. MN is rarely found in children, and the efficacy of imatinib on pediatric patients remain unclear.

CASE SUMMARY

We report 2 pediatric cases diagnosed with MN with eosinophilia and PDGFRB rearrangement who were treated with imatinib. Case 1 was a 1-year-old girl admitted to the hospital because of “abdominal distension with hyperleukocytosis for 3 mo”. She had leukocytosis, anemia, and eosinophilia (the absolute eosinophil count (AEC) was 8960/μL), and her fluorescence in situ hybridization (FISH) test revealed that PDGFRB rearrangement was detected in 70% of 500 interphase cells. Case 2 was a 2-year-old girl admitted to the hospital because of “recurrent fever and rashes for 1 mo”. Her blood cell count showed an AEC of 3540/μL. The FISH test revealed that PDGFRB rearrangement was detected in 71% of 500 interphase cells. Both patients were diagnosed as MN with eosinophilia and PDGFRB rearrangement. Imatinib was added into their treatment regimen. As expected, complete hematologic remission was achieved after 1 mo of treatment, and symptoms disappeared.

CONCLUSION

Although MN with eosinophilia and PDGFRB rearrangement usually occurs in adults, it can be found in children. The therapeutic benefits of imatinib in these 2 pediatric patients were consistent with its reported effects in adult patients.

Keywords: Myeloid neoplasm; Platelet-derived growth factor receptor beta rearrangement; Eosinophilia; Children; Imatinib; Case report

Core Tip: In the present report, we describe 2 pediatric patients diagnosed as myeloid neoplasm (MN) with eosinophilia and platelet-derived growth factor receptor beta (PDGFRB) rearrangement and reviewed the relative literature to analyze the clinical and therapeutic features of this rare clinical entity. Although MN with PDGFRB rearrangement rarely occurs in children, awareness should be increased for the possibility of this disease. Detection of the mutant gene by fluorescence in situ hybridization is necessary once the disease is suspected. Imatinib had a considerable effect on children, though the dose of imatinib is still unclear. Moreover, additional attention should be paid regarding the prognosis, life expectancy, side effects, and quality of life of these pediatric patients.