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Clinical manifestations, pathologic features, and diagnosis of nodular lymphocyte-predominant Hodgkin lymphoma

Authors
Peter M Mauch, MD
Jon C Aster, MD
Ann S LaCasce, MD
Section Editors
Arnold S Freedman, MD
George P Canellos, MD
Deputy Editor
Alan G Rosmarin, MD

INTRODUCTION

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is an uncommon subtype of Hodgkin lymphoma (HL). This disorder is differentiated from all other forms of HL, which are commonly referred to as classical Hodgkin lymphomas (CHL), by characteristic pathologic and clinical features.

NLPHL represents a more indolent disease than CHL, and is therefore managed uniquely. Since this disease is so uncommon, most information concerning treatment and outcome has come from reports of single institutions or pooled, multi-institutional retrospective analyses.

The epidemiology, pathogenesis, clinical presentation, pathology, diagnosis, and differential diagnosis of NLPHL are reviewed here. Treatment and prognosis of NLPHL are discussed separately as is the diagnosis of CHL. (See "Treatment of nodular lymphocyte-predominant Hodgkin lymphoma" and "Epidemiology, pathologic features, and diagnosis of classical Hodgkin lymphoma".)

EPIDEMIOLOGY

Approximately 5 percent of patients initially diagnosed with HL have the uncommon variant NLPHL [1]. The incidence of NLPHL in the United States and Europe has been stable at approximately 8 to 9 cases per 10,000,000 people per year [2,3]. For unknown reasons, an increase in incidence among children has been noted.

Approximately 75 percent of patients with NLPHL are male [2]. The age distribution has two peaks: one in children and one in adults, the latter with a median age of 30 to 40 years [4,5]. Unlike CHL, NLPHL is more common in the United States among African-Americans compared with Caucasians [2].

                         

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Literature review current through: Nov 2016. | This topic last updated: Tue Aug 23 00:00:00 GMT+00:00 2016.
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