title: Adult lymphoblastic lymphoma in Taiwan: an analysis of treatment results of 26 patients.
abstract: Lymphoblastic lymphoma (LBL) frequently affects young adults and usually presents 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 countries, no reports regarding treatment of this disease have as yet been reported. We herein report our treatment experience and verify the efficacy of the Stanford/Northern California Oncology Group (NCOG) protocol for this disease 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. Nineteen patients had an initial stage IV disease. Of the 23 cases in which immunological studies were performed, 20 proved to be of T cell lineage, 1 of B cell type, and the other 2 lacked both T and B markers. Three major chemotherapeutic regimens including prednisone, methotrexate, doxorubicin, cyclophosphamide, etoposide-mechlorethamine, vincristine, procarbazine, prednisone (ProMACE-MOPP), cyclophosphamide, hydroxydaunomycin, vincristine, prednisone (CHOP), and the Stanford/NCOG protocol were used to treat 3, 6, and 15 patients, respectively. Two other patients were treated with two different chemotherapeutic regimens, respectively. One patient was excluded for analysis because of initial treatment by surgery. The complete response (CR) rates 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 patients 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 marrow transplantation (BMT). Two of them obtained long-term disease-free survival. Two other patients in CR were treated with high-dose chemotherapy (HDCT) supported with autologous BMT and peripheral blood stem cell transplantation (PBSCT), respectively. The patient receiving HDCT with autologous PBSCT died of LBL relapse 6 months after transplantation. The other patient undergoing HDCT with autologous BMT died of fulminant hepatitis 5.5 months after 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 long-term remission for patients in an early stage of disease. For those patients with LBL in an advanced stage or in relapse, HDCT with allogeneic or autologous BMT is probably the treatment of choice.
authors.affiliation: Department of Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, Republic of China.
authors.forename: Y C
authors.initials: YC
authors.lastname: Chen
authors.forename: C L
authors.initials: CL
authors.lastname: Ho
authors.forename: W Y
authors.initials: WY
authors.lastname: Kao
authors.forename: J M
authors.initials: JM
authors.lastname: Hwang
authors.forename: L F
authors.initials: LF
authors.lastname: Sheu
authors.forename: T Y
authors.initials: TY
authors.lastname: Chao
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage* / Q000627:therapeutic use*; D001215:Asparaginase / Q000008:administration & dosage*; D016026:Bone Marrow Transplantation; D003520:Cyclophosphamide / Q000008:administration & dosage*; D004317:Doxorubicin / Q000008:administration & dosage*; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008297:Male; D008466:Mechlorethamine / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D008875:Middle Aged; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy* / Q000401:mortality / Q000628:therapy; D011239:Prednisolone / Q000008:administration & dosage*; D011241:Prednisone / Q000008:administration & dosage; D011344:Procarbazine / Q000008:administration & dosage; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D016019:Survival Analysis; D013624:Taiwan; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage*
chemical_list: D011344:Procarbazine; D008466:Mechlorethamine; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D011239:Prednisolone; D001215:Asparaginase; D011241:Prednisone; D008727:Methotrexate