Chin Med J (Taipei) 1998;61:S129.

Lymphoblastic Lymphoma--An Analysis of 27 Cases

Yeu-Chin Chen, Woei-Yau Kao, Ching-Liang Ho, Tsu-Yi Chao

Division of Hematology/Oncology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, Republic of China

Abstract

Background. Lymphoblastic lymphoma (LL) is a distinct high-grade non-Hodgkin's lymphoma with an aggressive behavior and a high mortality rate. It frequently affects yooung adults, presents with a mediastinal mass and with bone marrow involvement. To further characterize LL in Chinese patients, we analyzed our patients with regard to the clinical features, cell types, prognostic factors, response to treatment and therapeutic outcomes.

Methods. Twenty-seven cases of histologically proved LL in Tri-Service General Hospital dated from January 1981 to December 1996 were analyzed. The 3 principal chemotherapy regimens used were ProMACE-MOPP, CHOP and Standford-NCOG protocol. Four patients in late stage or in relapse underwent allogeneic bone marrow transplantation(allo-BMT). Two patients in remission were treated with high-dose chemotherapy supported with auto-logous BMT(AMBT) and peripheral blood stem cells transplantation(APBSCT), respectively.

Results. The age of these patients ranged from 5 to 73 years old with a median of 21. At initial presentation, a mediastinal mass was seen in 23(85%) patients and bone marrow involvement in 14(52%). Most patients had late stage disease (stage IV in 61% of patients). The immunophenotyping study of 25 cases with biopsies or bone marrow aspiration performed in our hospital all showed T cell lineage. The complete response rate by ProMACE-MOPP, CHOP, and Standford-NCOG regimens were 0%, 16% and 93% respectively. Two patients with high dose of chemotherapy supported with APBSCT died of disease-relapse 6 months after transplantation. Another one patient receiving high dose chemotherapy supported with ABMT died of fulminant hepatitis 5.5 months after transplantation. Two of four patients with allo-BMT achieved long-term survival and remained alive and were disease-free 51 and 86 months after BMT. One patient was lost follow-up, and the median survival time of 26 patients was 13 months.

Conclusion. Our data suggest that Standford-NCOG protocol is an effective chemotherapy for LL and may provide long-term remission for patients with early stage disease. For those patients with LL in advanced stage and in relapse, allo-BMT is probably the treatment of choice.

Keywords: Lymphoblastic lymphoma, chemotherapy, allo-BMT

[Chin Med J (Taipei) 1998;61:S129.]