Nodular Lymphocyte-Predominant Hodgkin Lymphoma: Update on Biology and Treatment

Nodular lymphocyte-predominant B-cell/Hodgkin lymphoma (NLPB/HL) accounts for roughly 5% of all Hodgkin lymphoma (HL) cases. In contrast to classic HL (cHL), the malignant cells in NLPB/HL lack CD30 expression but maintain a complete phenotype of mature B-cells including CD20 and IgG, but occasionally show IgD expression. The tumor cells are known as lymphocyte-predominant (LP) cells and grow in a T-cell and histiocyte rich microenvironment against a background of follicular dendritic cells. Transformation to large B cell lymphoma may occur and requires excisional lymph node biopsies. Most patients with NLPB/HL present in early stages. The disease usually has an indolent clinical course. Approaches applied in cHL result in very good outcomes with only limited excess mortality in comparison with the general population. Activity has also been demonstrated for rituximab-containing regimens commonly used in indolent B-cell non-Hodgkin lymphoma. The present article reviews pathological characteristics, treatment options and tools that might improve risk stratification in NLPB/HL.