Large granular lymphocytic leukemia (LGLL) is a lymphoproliferative disease, with LGL infiltration in peripheral blood and bone marrow, hepatosplenomegaly, and cytopenia. Both T-LGLL and CLPD-NK are indolent disease and share similar biology and clinical course, and treated under the same strategy. So the investigators put them together as LGLL. The investigators used TPM regimen (thalidomide + prednison + methotrexate ) to treat LGLL since 2013, and 18/20 patients (90%) obtained clinical response, including 80% complete response. Adverse events (AE) of grade 3 and above are rare and safe. Therefore, the investigators designed this multicenter clinical trial to validate the efficacy of the TPM regimen in symptomatic T-LGLL and CLPD-NK.
Because LGLL has continuously activated cytotoxic T lymphocytes, immunosuppressive therapy is the standard first-line therapy for T-LGLL and CLPD-NK. Previous studies showed that the overall response rate (ORR) of first-line oral immunosuppressants ranged from 21% to 85% (median: 50%). Both methotrexate and cyclosporine A are LGLL first-line treatment options, but the CR rate of methotrexate is only 21%, while the CR rate of CsA is less than 5%. There is insufficient evidence for the treatment of LGLL with prednisone and other glucocorticoids, but it can reduce RA-related inflammation and increase granulocyte levels. The TPM regimen was designed by the investigators. A pilot prospect observation showed that 18/20 (90%) patients obtained response, including 80% CR. This study is a prospective multiple center clinical trail to evaluate the efficacy of TPM regimen in the treatment of symptomatic LGLL. Eligible patients choose the initial treatment plan: thalidomide 50-100mg qn+ prednisone 0.5-1mg / kg qod +methotrexate 10mg / m2 / week. Four months is one course. Maximum three courses will be given if there is a response and thalidomide maintenance will be for another year.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
42
thalidomide 50-100mg daily at bedtime + prednisone 0.5mg/kg qod to 1mg/kg qd + methotrexate 10mg/m2 per week. 4 months one cycle, up to 3 cycles. After get partial remission, thalidomide maintenance will continue up to 2 years.
The First Affiliated Hospital of Guangxi Medical University
Nanning, Guangxi, China
RECRUITINGHenan Cancer Hospital
Zhengzhou, Henan, China
RECRUITINGTongji hopital, Huazhong University of Science and Technology
Wuhan, Hubei, China
RECRUITINGThe Second Xiangya Hospital of Central South University
Changsha, Hunan, China
RECRUITINGThe First Affiliated Hospital of Nanchang University
Nanchang, Jiangxi, China
RECRUITINGThe First Affiliated Hospital of Jilin University
Ch’ang-ch’un, Jilin, China
RECRUITINGXijing Hospital, Air Force Military Medical University
Xi’an, Shanxi, China
RECRUITINGInstitute of Hematology & Blood Diseases Hospital
Tianjin, Tianjin Municipality, China
RECRUITINGTianjin First Central Hospital
Tianjin, Tianjin Municipality, China
RECRUITINGComplete response (CR) rate of TPM regimen
Hb\> 120g / L，platelet\> 100×109 / L，ANC \> 1.5×109 / L），ALC\< 4×109 / L，peripheral LGL in normal（\< 0.5×109 / L）
Time frame: From date of TPM treatment until the date of complete response, assessed up to 100 months
Overall response (PR)
improvement in blood counts (ANC \> 0.5 × 10\^9/L; HGB increased by \>1 g/dL; PLT \> 50 × 10\^9/L), and the absence of required transfusions.
Time frame: From date of TPM treatment until the date of at least partial response, assessed up to 100 months
Progression-free survival (PFS)
the length of time during and after the treatment of LGLL
Time frame: From date of TPM treatment until the date of first documented progression or date of death from any cause, whichever came first, assessed up to 100 months
Duration of response (DoR)
the time from response to progression/death (P/D)
Time frame: From date of getting response until the date of first documented progression or date of death from any cause, whichever came first, assessed up to 100 months
overall survival
the length of the patients survival time
Time frame: From date of TPM treatment until the date of death from any cause, assessed up to 180 months
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