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

Stier JR, Vasquez RJ - Case Rep Oncol Med (2015)

Bottom Line: A three-year-old boy presented with an enlarging neck mass.The patient received cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) with rituximab and had excellent results.The clinical characteristics among patients at various ages are discussed with a special focus on the IgD-positive subtype.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Tulane University School of Medicine, New Orleans, LA 70112, USA ; Department of Pediatrics, Ochsner Health System, New Orleans, LA 70112, USA.

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.

No MeSH data available.


Related in: MedlinePlus

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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fig1: 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.

Mentions: PET/CT (Figure 1) demonstrated increased tracer uptake within bilateral cervical lymph nodes (estimated SUV of 4.5) and spleen (estimated SUV of 3.6). Bone marrow biopsies showed no morphologic evidence of metastatic disease. Staging was discussed with several international Hodgkin experts who agreed that splenic disease without involvement of thoracic or intra-abdominal lymph nodes would be unlikely in NLPHL particularly without focal areas of hypermetabolic activity within the spleen; however, we could not completely discount this finding and it factored into our therapy plan. B-symptoms are unusual in NLPHL and since the patient's systemic symptoms resolved with antibiotics and did not recur in the intervening weeks prior to initiation of chemotherapy, we concluded that the constitutional symptoms were likely due to an unrelated infectious process. The lymphoma was therefore staged as IIa.


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

Stier JR, Vasquez RJ - Case Rep Oncol Med (2015)

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.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4388010&req=5

fig1: 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.
Mentions: PET/CT (Figure 1) demonstrated increased tracer uptake within bilateral cervical lymph nodes (estimated SUV of 4.5) and spleen (estimated SUV of 3.6). Bone marrow biopsies showed no morphologic evidence of metastatic disease. Staging was discussed with several international Hodgkin experts who agreed that splenic disease without involvement of thoracic or intra-abdominal lymph nodes would be unlikely in NLPHL particularly without focal areas of hypermetabolic activity within the spleen; however, we could not completely discount this finding and it factored into our therapy plan. B-symptoms are unusual in NLPHL and since the patient's systemic symptoms resolved with antibiotics and did not recur in the intervening weeks prior to initiation of chemotherapy, we concluded that the constitutional symptoms were likely due to an unrelated infectious process. The lymphoma was therefore staged as IIa.

Bottom Line: A three-year-old boy presented with an enlarging neck mass.The patient received cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) with rituximab and had excellent results.The clinical characteristics among patients at various ages are discussed with a special focus on the IgD-positive subtype.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Tulane University School of Medicine, New Orleans, LA 70112, USA ; Department of Pediatrics, Ochsner Health System, New Orleans, LA 70112, USA.

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.

No MeSH data available.


Related in: MedlinePlus