Published: 21 September 2016. Authors:B. Eichhorst1, T. Robak2, E. Montserrat3, P. Ghia4, P. Hillmen5, M. Hallek6 & C. Buske7, on behalf of the ESMO Guidelines Committee
1Klinik I für Innere Medizin, Universität zu Köln, Köln, Germany; 2Department of Hematology, Medical University of Lodz, Copernicus Memorial Hospital, Lodz, Poland; 3Institute of Hematology and Oncology, Department of Hematology, Hospital Clinic, University of Barcelona, Barcelona, Spain; 4Department of Onco-Hematology and Division of Molecular Oncology, Università Vita-Salute San Raffaele, Fondazione Centro San Raffaele, IRCCS Istituto Scientifico San Raffaele, Milan, Italy; 5Institute of Oncology, St James’s University Hospital, Leeds, UK; 6Department I Internal Medicine, Center for Integrated Oncology Köln-Bonn, Center of Excellence on Cellular Stress Responses in Aging-Associated Diseases, University of Cologne, Köln; 7Comprehensive Cancer Center Ulm, Institute of Experimental Cancer Research, University Hospital, Ulm, Germany
This update refers to the Chronic lymphocytic leukaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up, Eichhorst B, Robak T, Montserrat P et al, Ann Oncol 2015; 26 (Suppl 5): v78-v84.
Treatment of advanced disease stage - Front-line treatment
The Btk inhibitor ibrutinib was superior to chlorambucil alone regarding PFS and overall survival in a phase III study including mostly elderly patients . If access is available, ibrutinib can be considered as an alternative treatment option to chlorambucil-based chemoimmunotherapy. However, lack of long-term experience with front-line therapy with ibrutinib must be taken into consideration [I, C].
Patients with TP53 deletion/mutation have a poor prognosis even after FCR therapy . Therefore, it is recommended that patients with TP53 deletion/mutation are treated with ibrutinib in front-line [V, A]. Because of severe infectious complications, the PI3K inhibitor idelalisib combined with rituximab is only recommended for frontline therapy in patients not suitable for Btk inhibitors, if anti-infective prophylaxis is taken and measures to prevent infection are followed. In the relapse setting, ibrutinib and idelalisib plus rituximab are treatment options.
Treatment of advanced disease stage - Treatment of CLL complications
Infections are a common complication in CLL patients, therefore use of immunosuppressive agents, as for example corticosteroids, should be restricted to a possible minimum. The use of prophylactic systemic immunoglobulin does not have an impact on OS [1, 2], and is only recommended in patients with severe hypogammaglobulinaemia and repeated infections [I, A]. Antibiotic and antiviral prophylaxis should be used in patients with recurrent infections and/or very high risk of developing infections (for example, pneumocystis prophylaxis with cotrimoxazole during treatment with chemoimmunotherapies based on purine analogues or bendamustine or during treatment with idelalisib plus rituximab) [IV, B]. Pneumococcal vaccination as well as seasonal flu vaccination is recommended in early stage CLL [IV, B]. Cytomegaly virus surveillance is recommended during treatment with idelalisib plus rituximab.