E. I. Dorokhina, T. N. Moiseeva, A. M. Kovrigina, I. A. Shupletsova, L. S. Al-Radi, Ya. K. Mangasarova, S. R. Goryacheva
National Research Center for Hematology, Moscow, Russian Federation
Introduction: NLPHL accounts for 5% of all Hodgkin's Lymphoma (HL) and is a morphologically heterogeneous type. The clinical significance of the presence of THRLBCL-like patterns in NLPHL is unknown and is the subject of scientific research.
Aim: To study the clinical features of NLPHL with the presence of THRLBCL-like patterns, to evaluate the efficacy of first-line therapy in this group of pts.
Materials and methods: During 2010–2017 75 patients (m/f 9: 2, median of age 37 years (18–69)) were observed at our center with NLPHL. The diagnosis and stage of the disease is based on the standard methods for HL. Presence of THRLBCL-like pattern was detected in 33 (44%) pts, including 9/33 (27%) pts with predominance of THRLBCL-like patterns in lymph node biopsies (more than 50% of the area of the sections).
Results: Advanced stages of disease was established in 30 (91%) pts; transformation into LBCL - in 2/33 (6%) pts with THRLBCL-like patterns of NLPHL. B-symptoms were present in 22 (67%) cases. Extranodal involvement - in 23 (70%) pts: liver - in 16 (48%), lung − 7 (21%), spleen − 17 (52%); bone marrow involvement - in 13 (40%) pts. R-ABVD therapy received 6/33 (18%) pts in a group with THRLBCL-like patterns NLPHL. All pts achieved complete remission (CR) of the disease. 24 (73%) pts received R-BEACOPP-14, 19 (79%) of them achieved CR, progression of the disease (PD) was detected in 5 (21%) pts, who received second-line therapy, two pts followed by ASCT. From the group with PD - 3/5 pts achieved CR, 1/5 continue therapy, 1/5 is now on palliative protocol due to therapy failure. From the general group of NLPLH with THRLBCL-like patterns 1/33 (3%) pts received R-CHOP with PR and PD after 1.5 years, currently -in CR by R-BEACOPP-14. 1/33 (3%) pt received R-DA-EPOCH with PD, second-line therapy is planned. 2/33 pts showed morphological and clinical evidence of transformation into LBCL, 1 pt with a relapse (after 31 years) died after 1 course ABVD, the second patient achieved CR by R-BEACOPP-14. A group of 9 pts with more than 50% THRLBCL-like patterns was characterized by an aggressive disease with extranodal lesion in 8/9 pts and a poor response to first-line therapy (4 pts needed second- line therapy).
Conclusion: Clinical features of pts with THRLBCL-like patterns in NLPHL are generalized lesions with B-symptoms, frequent extranodal and bone marrow involvement. Therapy by R-BEACOPP-14 and R-ABVD allows to achieve CR in 75% of pts.