Angioimmunoblastic T-cell lymphoma(AITL) is a rare,distinct subtype of peripheral T-cell lymphoma,possessing an aggressive course and poor prognosis with no standard therapy.Twelve patients who have failed at least tw...Angioimmunoblastic T-cell lymphoma(AITL) is a rare,distinct subtype of peripheral T-cell lymphoma,possessing an aggressive course and poor prognosis with no standard therapy.Twelve patients who have failed at least two initial CHOP or CHOP-like regimens were enrolled in this study and treated with individualized cyclosporine(CsA),prednisone(PDN),and monthly,high-dose intravenous immunoglobulin(HDIVIG).The dose of CsA was adjusted individually based on the blood trough concentration of CsA and renal function.All patients were examined for response,toxicity and survival.The most significant toxicities(≥ grade 2) were infection(16.7%),renal insufficiency(8.3%),hypertension(8.3%),diabetes(8.3%) and insomnia(16.7%).Discontinuation of treatment occurred in one patient(8.3%) due to grade 3 renal toxicity and subsequent grade 4 pulmonary infection.Treatment-related death was not observed.The overall response rate was 75.0%(complete response,33.3%;partial response,41.7%).With a median follow-up of 25.5 months,the median duration of response was 20 months(range,12 to 49 months) and the median progression-free survival(PFS) was 25.5 months(range,10 to 56 months).The 2-year PFS rate was 81.5%.Our findings indicate the combination of CsA,PDN and HDIVIG is an effective salvage regimen for refractory or relapsed AITL with predictable and manageable toxicity.展开更多
The histopathology, immunophenotype and clinical presentations of 24 cases of immunoblastic lymphadenopathy(IBL)-like T-cell lymphoma were studied. The results showed that fever and generalized lymphadenopathy were th...The histopathology, immunophenotype and clinical presentations of 24 cases of immunoblastic lymphadenopathy(IBL)-like T-cell lymphoma were studied. The results showed that fever and generalized lymphadenopathy were the first appeared and more commonly occurred clinical manifestations. 12 of 19 cases(63. 2%) which had been followed up died of relapse and progression of the disease.Pathologically, focus and/ or sheet- like proliferation of tumorous lymphoid cells, including pale cells and convoluted cells with obliteration of involved lymph nodes was the histologic feature of the disease. Within these 24 cases of IBL-like T-cell lymphomas diagnosed by morphologic criteria, tumor cells of 20 cases gave positive reaction to T-cellmarkers and the remaining 4 cases were reactive to B cell markers as well. Immunoblasts and plasma cells showed polyclonal proliferation. Immunotypic analysis showed that rearrangement of T-cell receptor beta chain was detected in 1 case of the disease. Pathologic diagnosis of the disease and relationships between biologic behaviors and the prognosis were also discussed.展开更多
文摘Angioimmunoblastic T-cell lymphoma(AITL) is a rare,distinct subtype of peripheral T-cell lymphoma,possessing an aggressive course and poor prognosis with no standard therapy.Twelve patients who have failed at least two initial CHOP or CHOP-like regimens were enrolled in this study and treated with individualized cyclosporine(CsA),prednisone(PDN),and monthly,high-dose intravenous immunoglobulin(HDIVIG).The dose of CsA was adjusted individually based on the blood trough concentration of CsA and renal function.All patients were examined for response,toxicity and survival.The most significant toxicities(≥ grade 2) were infection(16.7%),renal insufficiency(8.3%),hypertension(8.3%),diabetes(8.3%) and insomnia(16.7%).Discontinuation of treatment occurred in one patient(8.3%) due to grade 3 renal toxicity and subsequent grade 4 pulmonary infection.Treatment-related death was not observed.The overall response rate was 75.0%(complete response,33.3%;partial response,41.7%).With a median follow-up of 25.5 months,the median duration of response was 20 months(range,12 to 49 months) and the median progression-free survival(PFS) was 25.5 months(range,10 to 56 months).The 2-year PFS rate was 81.5%.Our findings indicate the combination of CsA,PDN and HDIVIG is an effective salvage regimen for refractory or relapsed AITL with predictable and manageable toxicity.
文摘The histopathology, immunophenotype and clinical presentations of 24 cases of immunoblastic lymphadenopathy(IBL)-like T-cell lymphoma were studied. The results showed that fever and generalized lymphadenopathy were the first appeared and more commonly occurred clinical manifestations. 12 of 19 cases(63. 2%) which had been followed up died of relapse and progression of the disease.Pathologically, focus and/ or sheet- like proliferation of tumorous lymphoid cells, including pale cells and convoluted cells with obliteration of involved lymph nodes was the histologic feature of the disease. Within these 24 cases of IBL-like T-cell lymphomas diagnosed by morphologic criteria, tumor cells of 20 cases gave positive reaction to T-cellmarkers and the remaining 4 cases were reactive to B cell markers as well. Immunoblasts and plasma cells showed polyclonal proliferation. Immunotypic analysis showed that rearrangement of T-cell receptor beta chain was detected in 1 case of the disease. Pathologic diagnosis of the disease and relationships between biologic behaviors and the prognosis were also discussed.
