Myeloid neoplasms with eosinophilia and abnormal PDGFRA/B: report of three cases and review of literature
10.3760/cma.j.issn.1009-9921.2018.12.010
- VernacularTitle:伴有嗜酸粒细胞增多和PDGFRA/B异常的髓系肿瘤三例并文献复习
- Author:
Roujia WANG
1
;
Xiaoxia HU
;
Mengqiao GUO
;
Shenglan GONG
;
Xiong NI
;
Shuqing LYU
;
Jianmin WANG
Author Information
1. 第二军医大学附属长海医院血液内科 全军血液病研究所
- Keywords:
Eosinophilia;
Gene fusion;
Myeloid neoplasms;
Remission
- From:
Journal of Leukemia & Lymphoma
2018;27(12):744-749
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate clinical and hematological features of myeloid neoplasms with eosinophilia and abnormal PDGFRA/B and the effect of imatinib. Methods The data of three eosinophilia patients with abnormal PDGFRA/B fusion gene in Changhai Hospital, the Second Military Medical University and 22 Chinese cases reported in Chinese medical journals were analyzed. Thirty-one cases of idiopathic hypereosinophilic syndrome from Changhai Hospital, the Second Military Medical University were used as the controls. Results Compared with idiopathic hypereosinophilic syndrome, no differences were found in age, percentage of bone marrow eosinophils and counts of platelets in peripheral blood in myeloid neoplasms with eosinophilia and abnormal PDGFRA/B (all P ＞0.05), but statistical differences were found in gender (χ2＝5.080, P ＝ 0.016), peripheral blood white blood cell count (t ＝ 4.908, P ＝ 0.001), eosinophilic granulocyte absolute value (χ2＝ 17.230, P ＝ 0.001) and hemoglobin concentration (t ＝ 2.770, P ＝ 0.013). The median follow-up time was 17 months (3-108 months) in 24 myeloid neoplasms patients with eosinophilia and abnormal PDGFRA/B from Chinese report. Complete hematopoietic remission (CHR) rate was 91.7 % (22/24) after the treatment of imatinib. The total complete molecular remission (CMR) rate was 75.0 % (18/24). The median time of remission was 3 months (1-8 months). CMR in patients with PDGFRA and with PDGFRB was 76.5 % (13/17) and 85.7 % (6/7), respectively. Only one patient (4.2 %) died of disease relapse. Conclusion Imatinib has a favorable effect on myeloid neoplasms with eosinophilia and abnormal PDGFRA/B featured by distinct hematologic and clinical manifestations.
