Fulminant Synchronous Epstein-Barr Virus-Positive MALT Lymphoma and Diffuse Large B-Cell Lymphoma Monomorphic Post-Transplant Lymphoproliferative Disorder in a Child Following Heart Transplant: A Case Report.

Abstract

Background: Post-transplant lymphoproliferative disorder (PTLD) is a devastating complication following solid-organ and hematopoietic stem cell transplantation. While PTLD is often effectively managed with immunotherapy or immuno-chemotherapy, rare cases present in a fulminant manner and are rapidly life-threatening.

Methods: We describe a case of an Epstein-Barr virus (EBV)-negative 16-year-old who developed EBV DNAemia and rapidly fulminant monomorphic PTLD within five months of receiving a heart transplant from an EBV-positive donor.

Results/important clinical findings: Three months after transplant the patient developed EBV DNAemia, cytopenias, and severe gastrointestinal symptoms that led to the diagnosis of monomorphic PTLD, EBV-positive extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). The patient underwent immunotherapy, followed by two different immuno-chemotherapy regimens, none of which slowed the rapid progression of the disease. One month after the initial diagnosis, a repeat biopsy revealed the emergence of a distinct monomorphic PTLD, EBV-positive diffuse large B-cell lymphoma (DLBCL). The patient died of disease 10 weeks after diagnosis.

Conclusions: The case exemplifies a fulminant case of EBV-positive MALT lymphoma as a form of monomorphic PTLD in a child. The case illustrates that MALT lymphoma may have an aggressive natural history, and the apparent rapid emergence of a distinct form of PTLD reinforces the need for repeat tissue sampling upon disease relapse or when the clinical behavior does not match the expectation.