Abstract
Hodgkin lymphoma (HL) is a relatively rare malignant disease of lymphatic tissue. It accounts for only 0.5% of all cancer cases with an incidence of 2.9 cases per 100 000 of men and women per year.
The typical curve of incidence has 2 peaks in developed industrial countries: Young people are most frequently affected (age at diagnosis is 20-35 years in 70% of patients); the second peak of the disease is at the age over 60 years, more frequently in men (3:2). Medical history frequently includes infectious mononucleosis, autoimmune diseases and immunosuppression.
Genetic factors (familiar occurrence) have probably additional impact on the disease. Currently, HL is a malignant disease with the highest rate of curability.
This success was achieved by is due to immeasurable and financially demanding efforts of haemato-oncological centres worldwide. Long-lasting remissions are achieved (according to the clinical stage and risk factors) in 85-95% of patients and their life expectancy is currently more than 40 years.
Late effects of treatment are coming to the fore, especially cardiotoxicity and secondary malignancies. Current research is focused on two goals: Reduction of late effects in cured patients (by reducing chemotherapy intensity and by reducing radiotherapy dose and extent) while maintaining excellent efficacy of first-line treatment: Positron emission tomography (PET) plays a central role in this effort.
The second goal is to rescue the otherwise tragic fate of patients with relapsed/refractory HL (RR HL). New drugs based on immunotherapy such as brentuximab vedotin- an antibody-drug conjugate and anti-PD1 check-point inhibitors (nivolumab and pembrolizumab) are increasing in importance alongside conventional chemotherapy and radiotherapy.