Background and Aims

NBS is a rare syndrome of chromosomal instability which predisposition to lymphomas. Due to delay of NHL diagnosis and treatment problems the outcome in these patients remains poor

W present 25 NBS patients complicated by NHL from one center

Methods

Retrospective analysis of NHL clinical course in NBS patients

Results

From 1997, 25 NBS patients (aged 3,8-23ys)with NHL were diagnosed and/or treated in Department of Oncology CMHI. Ten children developed DLBCL, 8 TLBL, 3 Burkitt, 4 peripheral T-cell lymphoma. Two patients were classified as stage II, 17 as III, 6 as IV. Time from symptoms to diagnosis was delayed in 17/25. Thirteen patients received chemotherapy for mature B-cell lymphoma (11 LMB 89/2001, 2 R-CHOP), 11 for lymphoblastic lymphoma (BFM 90, EURO-LB04), one girl with angio-immunoblastic T-cell lymphoma was treated with steroids and cyclosporine and the second with CHOP with Bortezomib. None of the patients complied with chemotherapy time schedule due to treatment related complications. Six patients underwent high dose chemotherapy and alloHSCT. Nineteen patients died: 7 of progression, 6 of relapse, 3 of second leukemia/lymphoma, 3 of treatment related complications. One patient died from fifth identical molecularly episode of DLBCL 17 years after first diagnosis. Six patients are alive, three of them underwent alloHSCT in first remission, one is currently under treatment and alloHSCT is planned

Conclusions

Delay of NHL diagnosis, treatment complications and consecutive events of lymphoid malignancies results in poor outcome of NBS patients. AlloHSCT procedure should be performed in each NBS patient with NHL in first remission