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
- Author:
Ying Kun CHEN
1
,
2
;
Wen Juan YU
3
;
Hui LIU
3
;
Ju Ying WEI
3
;
Wen Bin QIAN
3
;
Jie JIN
3
Author Information
1. The First Affiliated Hospital of Zhejiang University, Hangzhou 310003,China
2. The First People's Hospital of Xiaoshan District, Hangzhou 311200, China.
3. 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.
