Management of nodular lymphocyte-predominant Hodgkin lymphoma: recommendations and unresolved dilemmas

Abstract

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare malignancy of young adults characterized by an indolent and recurrent course. Although relapses or transformation to aggressive B cell lymphoma can occur decades after the primary diagnosis, the prognosis of NLPHL is relatively good, with as much as a 90% 10-year overall survival rate. The rarity of NLPHL makes it difficult to conduct multicenter prospective studies to establish separate guidelines for the diagnosis and treatment of this disease.

Therefore, the recommendations for the treatment of NLPHL have for many years been the same as for classic Hodgkin lymphoma, except for early stages without risk factors. The registration of anti-CD20 monoclonal antibody for the treatment of CD20-positive B-cell lymphomas has opened up new perspectives for NLPHL patients. Modern and accurate histopathological examinations as well as imaging diagnostics, especially positron emission tomography/computed tomography has allowed a more precise distinction to be made between the indolent NLPHL and the transforming-to-aggressive lymphoma forms. This review is intended to provide readers with the clinical features, course, outcome and methods of standard treatment in patients with NLPHL. The author in particular wishes to draw attention to unresolved issues regarding standard management and also the use of active surveillance, anti-CD20 immunotherapy, less aggressive regimens of chemotherapy, and indications for new treatment options.