A Rare Case of Refractory Nodal Marginal Zone Lymphoma in a Child, Successfully Cured by Allogeneic Stem Cell Transplant: A Case Report.

Pediatr Transplant

Department of Pediatric Hematology/Oncology and Bone Marrow Transplant, Ministry of National Guard and Health Affairs, Riyadh, Saudi Arabia.

Published: September 2024

AI Article Synopsis

  • Nodal marginal zone lymphoma (NMZL) is a rare subtype of non-Hodgkin lymphoma, making up about 2% of cases in both children and adults, often considered to be a mild disease but can be aggressive in adults.
  • A 31-month-old boy with refractory EBV-positive NMZL experienced extensive disease that did not respond to initial chemotherapy or Rituximab, prompting treatment with second-line chemotherapy and an allogeneic hematopoietic stem cell transplant (HSCT).
  • This case is significant as it is the first reported instance of a pediatric patient with refractory NMZL successfully treated with allogeneic stem cell transplant, indicating potential new treatment options for similar cases.

Article Abstract

Background: Nodal marginal zone lymphoma (NMZL) is a rare form of non-Hodgkin lymphoma (NHL), accounting for around 2% of all NHL in pediatrics and adults. It is considered the least common subtype of marginal zone lymphoma, which is rarely seen in pediatrics and is usually viewed as a mild disease. In adults, its presentation varies from mild to aggressive disease with multiple lymph node enlargement and systemic symptoms.

Case Report: Here, we report an interesting case of a 31-month-old boy who developed refractory EBV-positive NMZL with extensive disease. The tumor did not respond well to conventional chemotherapy and Rituximab. Therefore, we manage the disease with second-line chemotherapy, including bortezomib, followed by an allogeneic hematopoietic stem cell transplant (HSCT) with an excellent outcome.

Conclusion: Very few cases have been reported for aggressive NMZL children. The tumor was refractory to first line chemotherapy. However, the successful outcome was achieved by allogeneic stem cell transplant with myeloablative conditioning chemotherapy. The significance of this particular case is that this is the first reported case from pediatrics for refractory NMZL, which responded well to allogeneic stem cell transplant.

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Source
http://dx.doi.org/10.1111/petr.14835DOI Listing

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