The unique entity of nodular lymphocyte-predominant Hodgkin lymphoma: current approaches to diagnosis and management

Abstract

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare disease constituting only 3–8% of all Hodgkin lymphoma. It has a distinct histological and clinical presentation as well as significantly different natural history compared to the classical form of Hodgkin lymphoma. Presenting most often as early-stage disease, NLPHL is characterized by frequent relapses, but paradoxically an overall good prognosis. The approach to management should therefore reflect this pattern and focus on attaining prolonged remissions, with long-term follow-up paramount. Due to the rarity of the disease, few prospective data exist. Options for treatment include radiotherapy, chemotherapy or combined chemotherapy plus radiotherapy and targeted anti-CD20 antibody therapy, as well as observation in selected patients.

Keywords::

• Nodular lymphocyte-predominant Hodgkin lymphoma
• Hodgkin lymphoma
• chemotherapy
• radiotherapy
• rituximab

Acknowledgements

The authors acknowledge National Health Service funding from the National Institute for Health Research Biomedical Research Centre.

Potential conflict of interest: Disclosure forms provided by the authors are available with the full text of this article at www.informahealthcare.com/lal.