C. Afonso, A. Roque, D. Neves, A. Pinto, D. Mota, R. Guilherme, M. Gomes, L. Ribeiro
Clinical Hematology Department, Coimbra University Hospital Centre, Coimbra, Portugal
Background: Primary progressive Hodgkin Lymphoma (defined as progression during induction treatment or within 90 days of its completion) is associated with poor outcome, with long-term remission rate of only 30%. The treatment of choice for suitable patients (pts) is salvage chemotherapy followed by autologous stem cell transplant (ASCT).
Aims: Analyse risk factors and clinical outcomes of pts with primary progressive classical Hodgkin Lymphoma (PPcHL).
Methods: We performed a retrospective analysis of 389 cHL pts treated in a tertiary centre between 1990 and 2017. Univariate analysis was performed and significant predictors at the level of 0.05 were used to adjust a multivariate logistic regression model.
Results: We identified 53 (13.6%) pts with PPcHL, with a median age of 36 years (18–80) and homogeneous gender distribution. The most prevalent histological subtype was nodular sclerosis (75%). Most pts had intermediate or advanced disease (90.6%). We found an association between PPcHL and Ann Arbor stage III/IV (62.3 vs 46.5%; OR 2.14; p = 0.012), presence of B symptoms (71.7 vs 51.2%; OR 2.41; p = 0.007), bone marrow involvement (15.1 vs 6.3%; OR 2.67; p = 0.028) and serum albumin < 4 g/dL (OR 2.17; p = 0.014). No statistically significant association was found between PPcHL and the presence of mediastinal bulk, number of nodal regions, extranodal involvement or LDH>upper normal limit (p = NS). PPcHL was more common in pts with advanced disease by GHSG criteria (62.3 vs 43.2%; OR 2.17; p = 0.011) and in those with a positive interim FDG-PET (OR 7.39; p < 0.001). No association found with IPS≥3. On multivariate regression analysis no factor was predictor of developing PPcHL. Thirty-four (64.2%) pts received first line ABVD and 12 (22.6%) received consolidative radiotherapy. ESHAP was the most frequently used salvage regimen (52,5%); 14 pts (26.4%) underwent ASCT. The overall response rate (ORR) to salvage treatment in PPcHL was inferior to the ORR in pts that relapsed after 90 days (35.7% vs 52.7%; p = NS). PPcHL was associated with inferior OS (5-year OS: 51.8% vs. 88.8%, p < 0.001; 10-year OS: 44.4% vs 82.4%, p < 0.001) and had a negative impact on mortality (HR 4.12; p < 0.001).
Conclusions: In our cohort, GHSG criteria (but not IPS) seem to predict the risk for PPcHL. These patients had a poorer outcome when compared to patients that relapsed >90 days after completing therapy. It is important to identify high-risk patients at diagnosis and devise more effective treatment strategies.