Poor Outcome for Children and Adolescents with Progressive Disease or Relapse of Lymphoblastic Lymphoma - a Report of the BFM Group

Background/Objectives: Little is known about the outcome of pediatric patients with lymphoblastic lymphoma (LBL) who suffer form progressive disease or relapse.

Patients and Methods: We analyzed the pattern of LBL-relapses after current NHL-BFM-frontline therapy between 4/90 and 3/03. Relapse therapy was according to ALL-Relapse-BFM protocols or ALL-BFM protocols for high-risk patients.

Results: 28 of 251 registered T-LBL-patients (11%) and six of 73 pB-LBL-patients (8%) suffered from relapse. Of the 28 T-LBL-patients, one died from infection during relapse-chemotherapy, 18 failed to achieve stable remission and died from disease-progression, and nine reached allogeneic stemcell transplantation (SCT). Two of these nine SCT patients died from transplantation-associated toxicity, three died from disease-progression and four are still alive. The patients are in 2^nd remission of their lymphoma for 48, 68, 125 and 131 months respectively after allogeneic SCT. One of the four patients developed colon adenocarcinoma 47 months after SCT. Of the six relapsed pB-LBL patients one died due to toxicity of relapse-chemotherapy, two died from disease-progression after chemotherapy and three received allogeneic SCT. Of these, two died from subsequent disease-progression while one patient is still alive 57 months after allogeneic SCT.

Conclusions: Outcome of LBL-patients with relapse during or after current intensive 1^st line therapy is poor. More than 50% of these patients failed to achieve remission to intensive 2^nd line chemotherapy. Consolidation by allogeneic SCT may offer a cure for those who reach second remission.