Lymphocyte-Predominant Hodgkin's Disease in Children: A Case Study and Review of the Literature - PubMed (original) (raw)

Lymphocyte-Predominant Hodgkin's Disease in Children: A Case Study and Review of the Literature

James R Stier et al. Case Rep Oncol Med. 2015.

Abstract

A three-year-old boy presented with an enlarging neck mass. Biopsy demonstrated IgD-positive nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL), which was staged as IIa. The patient received cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) with rituximab and had excellent results. NLPHL is a relatively rare disease that is biologically distinct from classic Hodgkin lymphoma (cHL). NLPHL is a B-cell malignancy likely of germinal center origin that has an overall good prognosis and favorable response to treatment. Unlike cHL, NLPHL is ubiquitously CD20-positive. Recent evidence supports the efficacy of targeted anti-CD20 therapy in NLPHL, though prospective data is limited. This case demonstrates several unique features of NLPHL and further supports the use of rituximab in front-line therapy. The clinical characteristics among patients at various ages are discussed with a special focus on the IgD-positive subtype. A thorough literature search demonstrates this to be the youngest patient with NLPHL yet described.

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Figures

Figure 1

Figure 1

PET/CT demonstrates increased tracer uptake within bilateral cervical lymph nodes and the nasopharyngeal region (often normal in children). Slightly increased uptake in the spleen is seen in the image on the right with no apparent focal lesion.

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