Immunotherapy – Biomechanisms
H. Mociková1, A. Sýkorová2, J. Marková1, K. Klásková1, M. Šimkovic[Combining Caron]2, L. Smolej2, M. Kalinová3, V. Campr3, T. Kozák1, on behalf of the Czech Hodgkin Lymphoma Study Group
1 Department of Clinical Hematology, University Hospital Kralovske Vinohrady and Third Faculty of Medicine, Charles University, Prague, Czech Republic, 2 Fourth Department of Internal Medicine – Hematology, University Hospital and Faculty of Medicine, Hradec Kralove, Czech Republic, 3 Department of Pathology and Molecular Medicine, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, Czech Republic
Background: Transformation of B -chronic lymphocytic leukemia (B CLL) to Hodgkin lymphoma (HL) is a rare event. Optimal management of these patients (pts) is not established. This retrospective analysis summarizes diagnostics, treatment and prognosis of 10 pts with Hodgkin‘s variant (HV) of Richter syndrome in the Czech Republic.
Patients and Methods: Initial treatment of 7 pts with B CLL included fludarabine-based chemotherapy in combination with rituximab or alemtuzumab and 3 pts were treated with chlorambucil. HV of Richter syndrome was diagnosed in 10 pts (8 males) in the Czech Republic between 1996 and 2017. Median duration since diagnosis of B CLL till diagnosis of HV was 8.4 years (range 2.6 – 16.5). Median age at diagnosis of HV was 68 years (range 54 - 83). Identical clonal IgH rearrangement (VH7/DH1/JH6) from initial B CLL tissue and from HV cells was confirmed in only one patient, frozen tissues from other pts were either insufficient or unavailable. Seven pts with HV were treated with ABVD (doxorubicin, bleomycin, vinblastine, dakarbazine) including 2 pts in combination with rituximab. Another 3 pts received COPP (cyclophosphamide, vincristine, procarbazine, and prednisone). Involved field radiotherapy of 30Gy was used in 2 pts after chemotherapy.
Results: After treatment of Richter transformation to HV a complete remission was achieved in 3 pts, partial remission in 3 pts and progression in 4 pts. Only 4 pts are alive: 3 in longlasting complete remission and one patient in partial remission. Overall 6 pts died (3 in progression, one treatment toxicity, one solid tumour, one cause unknown). Median overall survival since diagnosis of B CLL was 207 months and since diagnosis of HV was 42 months.
Conclusion: Current treatment of Richter transformation to HV based on chemotherapy is not sufficient and these pts should be enrolled into clinical trials with new drugs.
This work was supported by grant AZV 16-29857A Ministry of Health of the Czech Republic and Research project Q28 Progres and Q40/08 Progres awarded by Charles University in Prague, Third Faculty of Medicine, Prague, Czech Republic