ABSTRACT
Due to disease rarity, there is limited information regarding the optimal therapy and outcome for patients with advanced-stage nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL). Forty-two patients with NLPHL by the Revised European-American Lymphoma/World Health Organization classification with advanced-stage disease were identified and paired 1:2 with a matched control with classical Hodgkin lymphoma (CHL) matched by age, gender, stage, decade of diagnosis, and treatment received. The median follow-up was 11.3 years (range, 1.9 to 35.5 years) for NLPHL patients and 10.7 years (range, 1.6 to 26.3 years) for CHL patients. The majority received doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD)-like chemotherapy. Although the 10-year overall survival (OS) (P = .579) and HL freedom from treatment failure (HL-FFTF) were similar between NLPHL and CHL patients (75% vs 73%; P = .610), the time to progression (TTP), which also includes the development of secondary aggressive lymphoma, was inferior in NLPHL (10-year, 63% vs 73%; P = .040). Splenic involvement was associated with an inferior 10-year TTP in patients treated with ABVD (48% vs 71%; P = .049) and an increased cumulative incidence of secondary aggressive lymphoma (P = .014) providing a rationale for further evaluation of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) with rituximab in NLPHL.
Subject(s)
Hodgkin Disease/drug therapy , Hodgkin Disease/pathology , Adolescent , Adult , Aged , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Case-Control Studies , Child , Female , Follow-Up Studies , Humans , Male , Matched-Pair Analysis , Middle Aged , Neoplasm Staging , Prognosis , Treatment Outcome , Young AdultABSTRACT
Splenic marginal zone lymphoma (SMZL) accounts for less than 2% of all non-Hodgkin lymphomas. We identified 107 cases diagnosed with SMZL between 1985 and 2012 from the British Columbia Cancer Agency Centre for Lymphoid Cancer and Lymphoma Pathology Databases. Patient characteristics were: median age 67 years (range 30-88), male 40%, stage IV 98%, splenomegaly 93%, bone marrow involvement 96%, peripheral blood involvement 87%. As initial treatment, 52 underwent splenectomy (10 with chemotherapy), 38 chemotherapy alone (21 chemoimmunotherapy containing rituximab, 1 rituximab alone), two antivirals for hepatitis C, and 15 were only observed. The 10-year overall survival for first-line splenectomy versus chemotherapy was 61% and 42%, respectively [Hazard Ratio (HR) 0·48, 95% confidence interval (CI) 0·26-0·88, P = 0·017]. The 10-year failure-free survival (FFS) after first-line splenectomy vs chemotherapy was 39% and 14%, respectively (HR 0·48, 95% CI 0·28-0·80, P = 0·004). Among the 38 patients who received first-line chemotherapy, FFS was similar between those receiving rituximab (n = 22) and those who did not (n = 16) (HR 0·64, 95% CI 0·31-1·34, P = 0·238). Fifteen patients transformed to aggressive lymphoma with median time to transformation of 3·5 years (range 6 months to 12 years) and the 10-year transformation rate was 18%. In conclusion, splenectomy remains a reasonable treatment for patients with SMZL.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Lymphoma, B-Cell, Marginal Zone/mortality , Lymphoma, B-Cell, Marginal Zone/therapy , Splenectomy , Splenic Neoplasms/mortality , Splenic Neoplasms/therapy , Adult , Aged , Aged, 80 and over , British Columbia/epidemiology , Disease-Free Survival , Female , Follow-Up Studies , Humans , Male , Middle Aged , Retrospective Studies , Survival Rate , Time FactorsABSTRACT
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) represents 5% of all Hodgkin lymphoma and has distinct clinico-pathological features. It is typified by the presence of lymphocyte predominant cells, which are CD20(+) , CD15(-) and CD30(-) and are found scattered amongst small B-lymphocytes arranged in a nodular pattern. Patients typically are males presenting with localized, peripheral lymphadenopathy. Despite frequent and often late or multiple relapses the prognosis is favourable. Deaths due to NLPHL are uncommon, but secondary malignancies and other treatment toxicities contribute appreciably to overall mortality. Secondary aggressive non-Hodgkin lymphoma can occur in approximately 7-14% of cases of NLPHL. Given this diseases' rarity, the optimal management is unclear and opinions differ as to whether treatment paradigms should be similar to or differ from those for classical Hodgkin lymphoma. This review provides an overview of the existing literature describing of the outcome and treatment approaches for limited and advanced stage NLPHL.