Nodular lymphocyte-predominant Hodgkin lymphoma: current management strategies and evolving approaches to individualize treatment

ABSTRACT

Introduction

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare lymphoma entity accounting for roughly 5% of all Hodgkin lymphoma (HL) cases. In contrast to classical HL, the malignant cells in NLPHL are positive for CD20 but lack CD30. The disease usually has an indolent clinical course resulting in high long-term survival rates.

Areas covered

In this review, treatment options for NLPHL are summarized and factors that may help to individualize treatment are discussed.

Expert opinion

Stage IA NLPHL without clinical risk factors should be treated with limited-field radiotherapy alone. In all other stages, NLPHL patients have excellent outcomes after standard HL approaches. The question of whether the addition of an anti-CD20 antibody to standard HL chemotherapy protocols or the use of approaches typically applied in B-cell non-Hodgkin lymphoma improve treatment results is unanswered until now. Different management strategies ranging from low-intensity treatment to high-dose chemotherapy and autologous stem cell transplantation have demonstrated activity in relapsed NLPHL. Second-line treatment is thus chosen individually. The major aim of NLPHL research is to spare toxicity and reduce the risk for treatment-related adverse events in low-risk patients while treating higher-risk patients with appropriate intensity. To this end, novel tools to guide treatment are required.

KEYWORDS:

• Nodular lymphocyte-predominant Hodgkin lymphoma
• radiotherapy
• chemotherapy
• anti-CD20 antibody treatment
• positron emission tomography
• histopathological growth pattern

Article highlights

• NLPHL has distinct pathological and clinical characteristics

• Treatment of NLPHL differs from classical HL in some situations

• Patients with NLPHL have an excellent overall prognosis

• Patients with variant growth patterns have an impaired outcome

• The histopathological growth pattern may play a role in future risk group allocation systems for patients with NLPHL

• The role of interim PET in NLPHL is not defined until now

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer Disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.