Wang Shi-Chong, Yang Wen-Yu
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.
World J Clin Cases. 2021 Jan 6;9(1):204-210. doi: 10.12998/wjcc.v9.i1.204.
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.
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 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.
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.
伴有嗜酸性粒细胞增多和血小板衍生生长因子受体β（PDGFRB）重排的髓系肿瘤（MN）在成人中对伊马替尼显示出良好的治疗反应。MN在儿童中很少见，伊马替尼对儿科患者的疗效仍不清楚。
我们报告2例诊断为伴有嗜酸性粒细胞增多和PDGFRB重排的MN的儿科患者，他们接受了伊马替尼治疗。病例1是一名1岁女孩，因“腹胀伴白细胞增多3个月”入院。她有白细胞增多、贫血和嗜酸性粒细胞增多（绝对嗜酸性粒细胞计数（AEC）为8960/μL），荧光原位杂交（FISH）检测显示，在500个间期细胞中有70%检测到PDGFRB重排。病例2是一名2岁女孩，因“反复发热和皮疹1个月”入院。她的血细胞计数显示AEC为3540/μL。FISH检测显示，在500个间期细胞中有71%检测到PDGFRB重排。两名患者均被诊断为伴有嗜酸性粒细胞增多和PDGFRB重排的MN。伊马替尼被添加到他们的治疗方案中。不出所料，治疗1个月后实现了完全血液学缓解，症状消失。
虽然伴有嗜酸性粒细胞增多和PDGFRB重排的MN通常发生在成人中，但也可在儿童中发现。伊马替尼在这2例儿科患者中的治疗效果与其在成人患者中的报道效果一致。
