Clinical analysis of myeloid neoplasms with t (3;21) (q26;q22).
10.3760/cma.j.issn.0253-2727.2019.03.006
- VernacularTitle:伴t(3;21)(q26;q22)髓系肿瘤临床分析
- Author:
Ye LI
1
;
Qing LIU
;
Zheng WANG
;
Ya Zhen QIN
;
Hui DANG
;
Yan SHI
;
Qi HE
;
Qian JIANG
;
Hao JIANG
;
Yue Yun LAI
Author Information
1. Peking University People's Hospital, Peking University Institute of Hematology, Beijing Key Laboratory of Hematopoietic Stem Cell Transplantation, Beijing 100044, China.
- Publication Type:Journal Article
- Keywords:
Leukemia, myeloid;
Translocation, genetic
- MeSH:
Adult;
Aged;
Chromosomes, Human, Pair 21;
Chromosomes, Human, Pair 3;
Female;
Humans;
Leukemia, Myeloid, Acute;
Male;
Middle Aged;
Myeloproliferative Disorders;
Retrospective Studies;
Translocation, Genetic;
Young Adult
- From:
Chinese Journal of Hematology
2019;40(3):195-199
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To analyze the characteristics of myeloid neoplasms with t (3;21) (q26;q22) . Methods: Clinical data of patients with t (3; 21) (q26; q22) , diagnosed as hematologic malignancies in Peking University people's hospital from January 2011 to March 2018, were collected retrospectively. 19 patients in our hospital and forty-eight patients bearing t (3;21) (q26;q22) with detailed survival data reported in literature were summarized. Kaplan- Meier method was used for survival analysis. Results: Among 19 patients, including 15 males and 4 females with a median age of 36 years (22-68 years) , 4 cases was diagnosed as de novo acute myeloid leukemia (AML) , 4 as myelodysplastic syndromes (MDS) , 3 as MDS-AML and 8 as chronic myelogenous leukemia (CML) in myeloid blast transformation. All of the 19 patients were detected to have t (3;21) (q26;q22) by G-banding technique and 13 carried additional cytogenetic aberrations. 9 of the 19 patients were detected for positive AML1-MDS1 fusion genes. In the 9 patients with detailed follow-up data, 6 patients received chemotherapy and only 2 achieved complete remission (CR) while 4 with no response. During the follow-up period, 8 patients died and the median overall survival (OS) was 6 months (4.5 to 22 months) . Survival analysis of the present 9 patients together with the literature data showed that the prognosis was poor and the median OS was 7 months. In particular, AML/t-AML had the worst prognosis. Hematopoietic stem cell transplantation (HSCT) could significantly improve survival, the median OS in HSCT group and non-HSCT group were 20.9 and 4.7 months respectively (P<0.001) . Conclusions: t (3; 21) (q26; q22) is a rare recurrent chromosomal abnormality which is detected mainly in myeloid neoplasm and confer to poor clinical prognosis. HSCT should be recommended to improve the outcomes.
