Aim. To describe clinical features of transformation of nodular Hodgkin's lymphoma (HL) with lymphoid predominance (NLHLP) to T-cell-rich large B-cell lymphoma (TCRLBCL), to evaluate the effectiveness of therapy program BEACOPP-14 in this group of patients.
Materials and methods. During the period from 2010 to 2016 the transformation NLHLP to TCRLBCL was diagnosed in 15 patients. Male to female ratio 4: 1, median age 40 (23-68) years. NLHLP diagnosis was based on histological and immunohistochemical study of the biopsy of the tumor, the stage of disease - by standard for LH methods.
Results. Of the 46 patients with NLPLH observed in National Reserch Center for Hematology, transformation NLHLP to TCRLBCL was diagnosed in 15 (33%) patients (13 untreated patients with a long history of lymphadenopathy, and 2 patients with relapsed Hodgkin's lymphoma after 3 years and 21 years ). All patients had advanced stages of disease. All untreated patients had previous long-term asymptomatic lymphadenopathy. B - symptoms were noted in 11 (73%) cases. Extranodal involvement had 12 (80%) patients: liver damage - in 7 (47%), lung involves - in 5 (33%), spleen involves - in 10 (67%), bone marrow lesions was found in 9 (60%) patients and was proved by histological and immunohistochemical study. Follow-up of the 15 patients: 1 patient with transformation to TCRLBCL in relapse (in 21year after the end of LH treatment) was not treated (refusal of treatment, death), 1 patient received ABVD and achieved complete remission, the remaining 13 patients received BEACOPP-14, complete remission was achieved in 11 (85%), partial remission - in 2 (15%) patients. In one young patient with transformation NLHLP to TCRLBCL with aggressive clinical presentation of disease complete remission was consolidated by high-dose chemotherapy followed by autologous peripheral blood stem cells transplantation.
Conclusions. The clinical features of transformation NLHLP to TCRLBCL are advanced stages of disease with presence of B-symptoms, frequent involvement of extranodal sites and bone marrow. Therapy by BEACOPP-14 program can lead to complete remission in 85% of patients.