Adult lymphoblastic lymphoma in Taiwan: an analysis of treatment results of 26 patients

Lymphoblastic lymphoma (LBL) frequently affects young adults and usually pr esents with a mediastinal mass as well as bone marrow involvement. Although the frequency of LBL in the Far East is higher than that of Western countr ies, no reports regarding treatment of this disease have as yet been report ed. We herein report our treatment experience and verify the efficacy of th e Stanford/Northern California Oncology Group (NCOG) protocol for this dise ase and recommend treatment strategies for LBL patients. We retrospectively reviewed the medical records of adult LBL patients treated in our hospital from 1986 to 1996. Twenty-seven patients were diagnosed to have LBL. These patients' ages ranged from 17 to 73 years old with a median of 23. Ninetee n patients had an initial stage IV disease. Of the 23 cases in which immuno logical studies were performed, 20 proved to be of T cell lineage, 1 of B c ell type, and the other 2 lacked both T and B markers. Three major chemothe rapeutic regimens including prednisone, methotrexate, doxorubicin, cyclopho sphamide, etoposide-mechlorethamine. vincristine, procarbazine, prednisone (ProMACE-MOPP), cyclophosphamide, hydroxy-daunomycin, vincristine, predniso ne (CHOP), and the Stanford/NCOG protocol were used to treat 3, 6, and 15 p atients, respectively. Two other patients were treated with two different c hemotherapeutic regimens, respectively. One patient was excluded for analys is because of initial treatment by surgery. The complete response (CR) rate s with ProMACE-MOPP, CHOP, and the Stanford/NCOG regimens were 0%, 17%, 80% and median overall survival 9, 8.5, and 15 months, respectively. Five pati ents with stage II-III diseases achieved long-term disease-free survival of 11-36 months with the Stanford/NCOG protocol with a median follow-up of 24 months. Four patients in late stage or relapse received allogeneic bone ma rrow transplantation (BMT). Two of them obtained long-term disease-free sur vival. Two other patients in CR were treated with high-dose chemotherapy (H DCT) supported with autologous BMT and peripheral blood stem cell transplan tation (PBSCT), respectively. The patient receiving HDCT with autologous PB SCT died of LBL relapse 6 months after transplantation. The other patient u ndergoing HDCT with autologous BMT died of fulminant hepatitis 5.5 months a fter transplantation. The median overall survival of all these 26 patients was 12 months. B symptoms and treatment without the Stanford/NCOG protocol were found to have significantly negative impacts on both patients' overall and progression-free survivals. Our results suggest that the Stanford/NCOG protocol may be an effective chemotherapy for adult LBL and may provide lo ng-term remission for patients in an early stage of disease. For those pati ents with LBL in an advanced stage or in relapse, HDCT with allogeneic or a utologous BMT is probably the treatment of choice.