Nodular lymphocyte-predominant hodgkin lymphoma

Abstract

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare lymphoma subtype accounting for about 5 % of Hodgkin lymphoma (HL) cases. It differs from the more common classical HL (cHL) in terms of histology, immunophenotype, and clinical course. In contrast to cHL, NLPHL is characterized by the presence of lymphocyte-predominant (LP) cells that express CD20, a hallmark of the disease, and consistently lack CD15 and CD30. Patients typically present with limited-stage disease affecting peripheral lymph nodes. The clinical course is significantly more indolent than in cHL, particularly in patients diagnosed with early-stage disease. For this group, treatment with involved-field radiotherapy (IF-RT) alone leads to excellent results. In more advanced stages, the standard of care is similar to cHL. Chemotherapy alone or combined-modality approaches are applied. Optimal therapy for relapsed NLPHL is undefined. Small trials with the anti-CD20 antibody rituximab report impressive response rates of 90-100 % with variable durability. In summary, NLPHL is an uncommon lymphoma entity often treated similar to cHL. However, different approaches appear possible due to the distinct immunophenotype and the more indolent clinical course.