Nodular Lymphocyte-Predominant Hodgkin Lymphoma: Review of Current Literature and Case Discussion

Abstract

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare subset of Hodgkin lymphoma (HL). It has a distinct clinical and pathological presentation. Unlike classic HL, where the predominant malignant cells are Reed Sternberg cells, the malignant cells in NLPHL are known as lymphocyte predominant (LP) cells, with their own unique immunohistochemistry antigen expression and staining pattern. Based on risk stratification and staging of the disease, treatment can range from active surveillance in asymptomatic patients with no organ compromise or bulky disease, to aggressive chemotherapeutic agents in advanced disease. Guidelines on which of these chemotherapy regimens would offer the most benefit to our patients are limited due to lack of randomized-controlled studies. Majority of the current prospective data on treatment were inclusive of both HL and NLPHL. Thus, the regimens employed in treatment of NLPHL are similar to the ones used in HL, though NLPHL is often viewed as its own distinct entity. This article aims to review the current literature and future advances on treatment of this rare disease.

Keywords: ABVD; Hodgkin lymphoma; NLPHL; RCHOP; lymphocyte predominant cells; nodular lymphocyte predominant Hodgkin lymphoma; ofatumumab; popcorn cells.

Image 1.

Hematoxylin and Eosin stained section from involved lymph node showing a typical “popcorn” cell characteristic of nodular lymphocyte predominant Hodgkin lymphoma. Note the multilobated nuclei and vesicular chromatin which is typically seen.

Image 2.

The tumor cells in nodular lymphocyte predominant Hodgkin lymphoma are strongly positive for several B-cell markers including CD20 and Pax-5 (seen here). In contrast Reed-Sternberg cells show very weak Pax-5 expression and are usually negative for CD20.

Image 3.

A large popcorn cell nuclei is positive for Oct-2 immunostain, this is a confirmatory finding in NLPHD (Nodular Lymphocyte Predominant Hodgkin Lymphoma).

Image 4.

Epithelial membrane antigen (EMA) immunostain is a useful stain in supporting the diagnosis of nodular lymphocyte predominant Hodgkin lymphoma. The neoplastic cells show membraneous and dot-like paranuclear staining in the Golgi region.

Image 5.

A useful diagnostic feature is the characteristic of PD1/CD279+ T-cells to form rosettes around the “popcorn” cells that characterize nodular lymphocyte predominant Hodgkin lymphoma. In this image the large atypical cell can be seen in the center surrounded by PD1 (+) T-lymphocytes.