Coexistent Kaposi sarcoma and post-transplant lymphoproliferative disorder in the same lymph nodes after pediatric liver transplantation: A case report

BACKGROUND: Kaposi sarcoma and post-transplant lymphoproliferative disorder have been occasionally reported in post-liver transplant patients. However, the simultaneous occurrence of these two diseases in the same lymph nodes is very rare. CASE SUMMARY: We report the case of a 19-mo-old boy, who presented with intermittent fever and enlarged cervical lymph nodes after liver transplantation. Six cervical lymph nodes were biopsied, and the histopathological examinations revealed multifocal hyperplasia of spindle cells around small blood vessels, extravasated erythrocytes, and heavy infiltration of plasma cells in the cortex and medulla of the lymph nodes. The immunohistochemical analyses of spindle cells revealed positive expression of CD34, CD31, erythroblast transformation-specific-related gene, friend leukemia integration 1, and human herpesvirus-8. The lymphoproliferative lesions expressed CD38, CD138, and multiple myeloma 1. Epstein-Barr encoded RNA in situ hybridization demonstrated Epstein-Barr virus-positive lymphoid cells. Finally, we diagnosed the coexistence of Kaposi sarcoma and post-transplant lymphoproliferative disorder (plasmacytic hyperplasia) in the same lymph nodes. Treatment strategy included anti-CD20 monoclonal antibody (rituximab) and discontinuation of the immunosuppressant therapies. Lymph node biopsies during follow-up examinations revealed lymphoid hyperplasia. CONCLUSION: The rare coexistence of Kaposi sarcoma and post-transplant lymphoproliferative disorder in the same lymph nodes post-liver transplantation possibly associates with immunodeficiency and Epstein-Barr virus and human herpesvirus-8 coinfection.