Clinical and laboratory analysis of 17 patients with γδT-cell large granular lymphocyte leukemia.
10.3760/cma.j.issn.0253-2727.2020.02.005
- Author:
Yang Min ZHU
1
;
Qing Yan GAO
;
Jing HU
;
Xu LIU
;
Dong Rui GUAN
;
Feng Kui ZHANG
Author Information
1. Department of Therapeutic Center of Anemia, Institute of Hematology & Blood Diseases Hospital, CAMS & PUMC, National Clinical Research Center for Blood Diseases, Tianjin 300020, China.
- Publication Type:Journal Article
- Keywords:
Cyclosporin;
Leukemia, T-cell large granular lymphocyte;
γδT-cell
- MeSH:
Adult;
Aged;
Humans;
Leukemia, Large Granular Lymphocytic;
Middle Aged;
Phenotype;
Retrospective Studies;
T-Lymphocytes
- From:
Chinese Journal of Hematology
2020;41(2):112-116
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To compare the difference of the clinical and laboratory characteristics between γδ T-cell large granular lymphocyte leukemia (γδT-LGLL) and αβ T-cell large granular lymphocyte leukemia (αβT-LGLL) . Methods: The clinical and laboratory characteristics of 17 patients with γδT-LGLL and 91 patients with αβT-LGLL in the department of therapeutic center of anemia of enrolled in our hospital from January 2009 to January 2019 were retrospectively analyzed. Results: The median age of the 17 patients with γδT-LGLL was 54 years (range, 25-73 years) , the most common presenting symptom was anemia. In comparison with αβT-LGLL patients, splenomegaly was common (41% and 44%, respectively) , whereas hepatomegaly (12% and 5%, respectively) and lymphadenopathy (6% and 8%, respectively) were rare. The positive rates of antinuclear antibody (59% and 45%, respectively) were high, whereas the positive rates of rheumatoid factor (6% and 10%, respectively) were rare for both groups. There were no differences on peripheral blood counts between the two groups. However, γδT-LGLL patients were found to be predominantly expressed a CD4(-)/CD8(-) phenotype. Steroid therapy with prednisone was used alone as first-line therapy for 1 patient. Cyclosporin A (CsA) was used alone as first-line therapy for 3 patients. CsA in combination with steroids were administered in 13 patients. After 4 months treatment, 2 patients acquired complete response, 4 patients acquired partial response, the overall response was 35%. Conclusion: γδT-LGLL is a rare mature T-lymphocyte proliferative disease. Clinical and laboratory characteristics were quite similar for γδT-LGLL in compare with αβT-LGLL. γδT-LGLL predominantly expressed a CD4(-)/CD8(-) phenotype. The data presented here indicate the CsA is an effective option for the first-line treatment of γδT-LGLL.
