Abstract:

Objective To investigate the clinical characteristics, treatment outcomes, and prognosis of hepatosplenic γδ T-cell lymphoma (HSγδTCL) in pediatric patients. Methods The clinical data of two pediatric patients with HS γδ TCL admitted to the oncology department of our hospital from October 2012 to May 2023 were retrospectively analyzed. Additionally, relevant literature was systematically searched and reviewed. Results The two patients were 9- and 11-year-old boys who presented with splenomegaly and cytopenia. Both were diagnosed with HSγδTCL. Patient 1 received chemotherapy but succumbed to disease progression. Patient 2 underwent seven cycles of chemotherapy in combination with histone deacetylase inhibitor therapy. After achieving remission, the patient received allogeneic hematopoietic stem cell transplantation and remains alive to date. A total of 30 pediatric cases were reviewed from the literature. Eight patients had a history of chronic immunodeficiency diseases and had received immunosuppressive therapy. Eleven patients underwent intensive chemotherapy combined with stem cell transplantation, while five patients had splenectomy followed by chemotherapy. Overall, 22 patients died, with a median survival time of less than one year after diagnosis. Conclusion HSγδTCL in children has a poor treatment outcome. Chemotherapy combined with hematopoietic stem cell transplantation is an alternative treatment strategy worthy of consideration.

Key words: hepatosplenic γδ T-cell lymphoma, child, chemotherapy, hematopoietic stem cell transplantation