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KEY CONCEPTS

  • Nodular lymphocyte–predominant Hodgkin lymphoma (HL) usually presents in early stages. Therapy depends on disease extent, but localized radiotherapy is the usual choice for early-stage disease. Rituximab is also an effective agent, but it should be given in combination with chemotherapy.

  • Staging by positron emission tomography is standard, and marrow biopsy is generally unnecessary. Response after two cycles of therapy is a standard to assess response, and the Lugano (Deauville score) response criteria should be followed. Patients with DS 1 or 2 should be considered as having a metabolic complete response. Those with DS 4 or 5 should be considered to have likely had failure of therapy, and those with persistent positivity after four cycles of therapy are at a high risk of failure. German Hodgkin Lymphoma Study Group criteria and the International Prognostic Score should be calculated for all patients to assess outcomes of therapy.

  • Standard of care for favorable early-stage classical HL is two cycles of ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) and 20-Gy involved site radiation therapy (ISRT). For unfavorable early-stage disease, it is four cycles of ABVD and 30 Gy ISRT. The ABVD regimen remains the preferred regimen for most patients with advanced disease treated in the United States even though BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone) has proven to be a better regimen for unfavorable early-stage and advanced-stage disease. BEACOPP is associated with a higher risk of neutropenia and hospitalization and in the United States is administered to only selected patients.

  • Brentuximab vedotin (BV) has been studied as adjuvant therapy for early-stage disease, as a single agent and in combinations for relapsed and refractory disease, as prophylaxis after autologous stem cell transplant (ASCT), and in combinations for initial treatment of classical Hodgkin disease. The drug causes some peripheral neuropathy but is generally tolerable, and the A (BV)-AVD regimen provides better progression-free survival results for most patients with advanced disease than ABVD.

  • PD-1 (programmed death-1) inhibitors (nivolumab, pembrolizumab) are also effective single agents for relapsed disease and in combinations improve responses for relapsed and untreated disease even in patients who have previously received BV. The most effective combinations using this and other targets are still under investigation.

  • High-dose therapy followed by ASCT remains the standard of care for patients with relapsed HL. However, proof of adequate response, usually by PET and in most patients by biopsy, is necessary before this therapy. Patients with refractory disease should be offered alternate therapies, and novel agents and therapies, including T-cell and other immunotherapy, are under investigation for this small group of patients.

INTRODUCTION

Hodgkin lymphoma (HL) was recognized in the first half of the 19th century by Thomas Hodgkin and Samuel Wilks.1 HL usually arises in lymph nodes, preferentially in the cervical area, and the majority of HLs manifest clinically in young adults in their third and fourth decades of life. In 2018, 79,990 new cases of the disease were described, and 34,984 ...

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