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Published on: June 10, 2017
Primary Splenic Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL): First Reported Case in a Young Child
Josselyn Sofia Vergara1, Lianna Jean Marks2, Oscar Silva1
1Department of Pathology, Stanford University School of Medicine, Stanford, CA, USA.
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) can rarely present primarily in the spleen in children. This case report details a child with primary splenic NLPHL who achieved disease-free status 3 years after splenectomy without further treatment.
Area of Science:
- Hematology
- Pediatric Oncology
- Pathology
Background:
- Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare B-cell lymphoma, typically indolent and affecting lymph nodes.
- Splenic involvement in NLPHL often indicates advanced disease and a poorer prognosis.
- Primary splenic NLPHL, especially in pediatric cases without nodal involvement, is exceptionally uncommon.
Purpose of the Study:
- To report the first known case of primary splenic Nodular lymphocyte predominant Hodgkin lymphoma in a child.
- To highlight the potential for favorable outcomes in pediatric primary splenic NLPHL following splenectomy.
Main Methods:
- Case report detailing a pediatric patient diagnosed with primary splenic NLPHL.
- Review of clinical presentation, diagnostic findings, treatment (splenectomy), and follow-up.
- Pathological confirmation of NLPHL in splenic tissue.
Main Results:
- The patient presented with isolated splenic involvement, lacking overt nodal disease.
- Following splenectomy, the child remains disease-free at 3-year follow-up without requiring additional therapy.
- This outcome contrasts with the generally poorer prognosis associated with splenic involvement in NLPHL.
Conclusions:
- Primary splenic NLPHL in children, though rare, can have an excellent prognosis.
- Splenectomy alone may be a sufficient treatment for pediatric primary splenic NLPHL.
- This case expands the understanding of NLPHL presentation and management in pediatric populations.
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