A. Visentin1, S. Pravato1, E. Scomazzon1, S. Imbergamo1, A. Branca1, M. Gregianin2, S. Vio3, C. Boso4, F. Piazza1, F. Vianello1, R. Zambello1, G. Semenzato1, L. Trentin1
1 Hematology and Clinical Immunology unit, Department of Medicine, University of Padua, Padua, Italy, 2 Nuclear Medicine Unit, Hospital of Castelfranco Veneto, Italy, 3 Radiology Unit, University Hospital of Padua, Padua, Italy, 4 Radiotherapy Unit, Veneto Institute of Oncology IOV-IRCSS, Padua, Italy
Background: Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) is an uncommon disease accounting for 5–10% of all Hodgkin lymphomas (HL). This subtype displays peculiar clinical, and biological characteristics as compared with classical HL. Different therapeutic approaches, ranging from watchful waiting (WW) to chemoimmunotherapy, are used to treat NLPHL. The outcome is generally favorable with low incidence of relapse or transformation to aggressive lymphoma.
We investigated the clinical features and therapeutic outcomes of patients with NLPHL followed at Padua university hospital.
Methods: All subjects with a diagnosis of Hodgkin's lymphoma referred to our Centre from January 2000 were retrospectively considered for the study. Histological data were reviewed together with age at diagnosis, gender, stage, systemic symptoms, first and further treatments, adverse events, relapse, death, date of last follow-up. FDG PET-TC scans were performed at diagnosis, cycle 2 (iPET) and at the end of treatment, and reported according to the Deauville 5-point scoring system (DS). Progression free survival (PFS) was calculated as time from diagnosis to progression or death, or last know follow-up. P values <0.05 were considered significant.
Results: Twenty-eight out of 338 HL patients fulfilled histological criteria of NLPHL, with a M/F ratio of 1.5 (17 males, 61%), median age of 50 years and 5 subjects older than 65 years. Twenty patients (71%) presented at an early-stage of disease (8 stage I and 12 stage II). After a median follow-up of 4.3 years for the whole population, 6 subjects relapsed and the median PFS was 11 years.
Rituximab was combined with ABVD in 18 patients (17 treatment naive and 1 relapsed, 33% stage III-IV); 9 subjects also received consolidative RT. After a median follow-up of 43 months, only 2 patients treated with R-ABVD relapsed and 1 transformed in high-grade lymphoma but none died. The median PFS was not reached and the estimated 5 and 10-year PFS were 90% and 68%, respectively. When considering iPET, 2 subjects (11%) had a DS ≥ 3 (iPET+) and among them, 1 relapsed after 7 years. Grade 3 or above adverse events were reported in 13 patients, including 4 rituximab infusion-related reactions, 13 neutropenias, 2 bleomycin pneumonitis, 1 infection and 1 nausea.
Conclusion: NLPHL is a rare disease which lack of standardized therapies. Data derived from our experience provide evidence of efficacy of rituximab combined with ABVD.