SeminarClassical Hodgkin lymphoma
Introduction
Although up to 90% of patients with classical Hodgkin lymphomas at all stages can now be cured, what causes the normal B-lymphocytes to become a malignant biologically active tumour cell is still unknown. Classical Hodgkin lymphoma accounts for 10–15% of all lymphomas and is a unique disease with specific clinical features, evolution, and response to therapy.
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Epidemiology and pathophysiology
Classical Hodgkin lymphoma has been recognised since 1832, when Thomas Hodgkin described the condition as occurring in young adults presenting with enlarged cervical nodes, weight loss, and fever, leading to death in a few months. Dorothy ReedMendenhall in 1898 and Carl Sternberg in 1902 found the typical Reed-Sternberg cell in microscopic analyses of these nodes.1 Classical Hodgkin lymphoma is a malignancy of the lymphatic system with an incidence of 2–3 cases per 100 000 people per year in
Staging
In contrast to other lymphomas, involved lymph-node chains in the vast majority of patients with classical Hodgkin lymphoma could be considered as contiguous by the criterion of direct lymphatic communication.11 The European Society for Medical Oncology guidelines for staging and risk assessment of classical Hodgkin lymphoma recommend a chest x-ray and a contrast-enhanced CT scan of the neck, chest, and abdomen, and a whole-body fluorodeoxyglucose (FDG)-PET scan for staging and response
Response assessment and follow-up
PET-CT is recommended for early-response (interim PET) and end-of-therapy assessment.13 In 2009, the Deauville 5-point scale was proposed for defining simple and reproducible criteria for the interpretation of the PET-CT images done early during treatment, for which visual assessment was not adequate.21 This scale defines the areas of residual FDG uptake in the disease compared with physiological mediastinal blood-pool and liver uptake (table 1). The optimal timepoint for interim PET-CT has
First-line treatment
The therapeutic approach for adults with classical Hodgkin lymphoma should be risk-adapted and depends on stage at presentation, clinical prognostic factors, and comorbidities.24 The Ann Arbor staging system11 remains in widespread use (panel 2), and two clinical scoring systems might be used to assign patients to treatment of various intensity (panel 3).1 Improvement of progression-free survival in patients with classical Hodgkin lymphoma from historical treatment to current therapies are
Management of relapsed or refractory disease
Approximately 10–25% of patients with classical Hodgkin lymphoma will have refractory disease or will relapse after achieving a complete remission. A biopsy to confirm classical Hodgkin lymphoma histology should be obtained whenever possible, especially in patients with primary progression to exclude composite lymphoma that has not been recognised at the initial diagnosis. The diagnosis of a non-Hodgkin lymphoma at relapse would have a serious impact on salvage therapy. A whole-body CT scan
Long-term follow-up
Survivors of classical Hodgkin lymphoma need to be followed not only for surveillance of disease recurrence but also for late effects of treatment, particularly secondary malignancies and cardiac toxicity.71 In an analysis of survivors of classical Hodgkin lymphoma treated with older radiation therapy techniques, 5-year survival after development of a second malignancy was 38%, and the excess risk of second malignancy continues to be elevated after 15–20 years of completing classical Hodgkin
Conclusion
Classical Hodgkin lymphoma is a unique disease recognised for more than 100 years that occurs mostly in young people. It represents a major success in cancer treatments with cure rates reaching 90% in 2020. The aim in recent years was to tailor treatment to prognostic factors to avoid long-term toxicity (eg, infertility and second cancers), which was mostly related to chemotherapy (alkylating agents) and extended fields of radiotherapy. For this purpose, the ABVD regimen has been widely used
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