Clinical characteristics and prognostic factors of 40 cases of primary systemic anaplastic large cell lymphoma.
10.3760/cma.j.issn.0253-2727.2020.03.007
- VernacularTitle:原发系统型间变性大细胞淋巴瘤40例临床特征及预后分析
- Author:
Ying Kun CHEN
1
;
Wen Juan YU
2
;
Hui LIU
2
;
Ju Ying WEI
2
;
Wen Bin QIAN
2
;
Jie JIN
2
Author Information
1. The First Affiliated Hospital of Zhejiang University, Hangzhou 310003,China; The First People's Hospital of Xiaoshan District, Hangzhou 311200, China.
2. The First Affiliated Hospital of Zhejiang University, Hangzhou 310003,China.
- Publication Type:Journal Article
- Keywords:
Anaplastic large cell lymphoma;
Anaplastic lymphoma kinase;
Clinical features;
Prognostic factors
- MeSH:
Adolescent;
Adult;
Aged;
Anaplastic Lymphoma Kinase;
Antineoplastic Combined Chemotherapy Protocols;
Cyclophosphamide;
Doxorubicin;
Female;
Humans;
Lymphoma, Large B-Cell, Diffuse;
Lymphoma, Large-Cell, Anaplastic;
Male;
Middle Aged;
Prednisone;
Prognosis;
Receptor Protein-Tyrosine Kinases;
Retrospective Studies;
Vincristine;
Young Adult
- From:
Chinese Journal of Hematology
2020;41(3):222-227
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To analyze the clinical features and prognostic factors of primary systemic anaplastic large cell lymphoma (ALCL) . Methods: 40 ALCL cases treated in the First Affiliated Hospital of Zhejiang University from January 2013 to December 2018 were retrospectively analyzed. Results: ① With a median age of 41 (14-67) years, there were 29 males and 11 females, 36 patients (90.0%) had Ann Arbor stage Ⅲ-Ⅳ tumors, 23 patients (57.5%) were in high-intermediate or high international prognostic index (IPI) risk group. 25 patients (62.5%) had B symptoms, such as fever, emaciation and night sweat.38 patients (95.0%) had extranodal invasion, 25 patients (62.5%) had higher LDH level, and 25 patients (62.5%) had high expression of Ki-67 (80% or more) . With 22 ALK(+) patients (55.0%) and 18 ALK(-) patients (45.0%) , there was a significantly difference in the median age of the two groups [29 (14-67) years old vs 51.5 (19-67) years old, P=0.003]. ② All patients received chemotherapy, 18 cases were treated with CHOP (cyclophosphamide, doxorubicin, vindesine, prednisone) , 12 cases with ECHOP (cyclophosphamide, doxorubicin, vindesine, prednisone, etoposide) , 10 cases with other treatments and 26 patients (65.0%) obtained complete remission (CR) . ALK(-) (P=0.029, OR=13.458) and Ki-67 expression of 80% or more (P=0.04, OR=14.453) were independent factors of CR rate, the CR rate of ECHOP chemotherapy was higher than CHOP chemotherapy (P=0.026) . ③ LDH level, IPI score, ALK expression and chemotherapy regimen had significantly effect on progression free survival (PFS) and overall survival (OS) (P<0.05) . Conclusion: The study shows that primary systemic ALCL usually occurs in males, the average age of ALK(+) patients were younger than ALK(-) patients. Most patients are in stage Ⅲ-Ⅳ with extranodal invasion, more than half of the patients have B symptoms, elevated LDH, and high expression of Ki-67. The expression level of Ki-67, ALK expression, and chemotherapy regimen have prognostic value for CR rate, the LDH level, IPI score, ALK expression and chemotherapy regimen for PFS and OS. ECHOP is a better choice with improved prognosis.
