Demographic insights in systemic EBV-positive T-cell lymphoproliferative disease of childhood: A comprehensive NCDB analysis.
Authors: Abi Karam Karam Rabih , Demangone Michael , Grover Jasika , Lee Daniel , Venkatesan Sharanya , Dieu Alexandria , Hsia Beau , Langland Dylan , Cronen Austin , Heise Craig , Silberstein Peter T. 
Published: June 2024

e19077

Background: Infection with the Epstein-Barr virus can result in lymphoproliferative disorders due to the capability of the virus to infect lymphocytic cells. Systemic EBV-positive T-cell lymphoproliferative disease (LPD) of childhood is a specific lymphoproliferative disorder caused by infection with the Ebstein-Barr virus that is characterized by T cell clonal proliferation that showcases cytotoxic T lymphocytes cells expressing a combination of the glycoproteins CD8 and CD4. The rarity of this lymphoproliferative disorder makes it a great candidate for further analysis of demographic factors impacting the disease. We will employ the National Cancer Database (NCDB) to shed light on the demographic complexities. Methods: A retrospective cohort analysis was conducted to examine all patients within the National Cancer Database (NCDB) who possessed histologically confirmed systemic EBV positive T cell LPD between 2004 and 2020. Descriptive statistical methods were applied to assess demographic factors, and a thorough regression analysis was employed to investigate patterns in the incidence of the disease. Results: The NCDB confirms a total of 91 patients from 2004-2020 having a diagnosis of systemic EBV-positive T-cell LPD of childhood. The average age of diagnosis was 28 years (IQR= 22). The majority of patients did not receive palliative care (98%). Primary treatment that patients received for this disease were chemotherapy (77%), hormone therapy (48%), immunotherapy (27%), and radiation therapy (7%). The two-year survival rate was 49% and the five-year survival rate was 46%. The mean survival time was around 5.6 years with this disease. Males and females were close to equal in numbers (52% and 48%). 69% of patients were white, 13% were black, and 82% were non-Spanish; non-Hispanic. A greater percentage of patients were privately insured (59%) compared to patients insured through Medicare or Medicaid. The primary site was bone marrow (47%) and the NCDB analytic stage group was high at Stage IV (58%). The majority had Charlson-Deyo comorbidity scores of 0 (84%). Conclusions: Following a thorough investigation of existing literature, we conclude this is the first NCDB analysis on systemic EBV-positive T-cell LPD of childhood, bridging a critical knowledge gap in the current research landscape. Socioeconomic factors have yet to be discussed revolving patients with systemic EBV-positive LPD of childhood. Our study showed a higher prevalence of the disease was found in privately insured, white patients with few comorbidities. Considering the mean age of diagnosis and prognosis of this disease, it is imperative that further research be conducted to understand the interaction between socioeconomic and demographic characteristics on the diagnosis, treatments, and survival of patients living with this cancer.