Multifocal cutaneous and systemic plasmablastic lymphoma in an infant with combined living donor small bowel and liver transplant

Abstract:  Small bowel allograft recipients have a relatively high risk (approximately 20%) of developing PTLD. Onset of PTLD is usually soon after transplant (median of eight months). Children are at a higher risk than adults. Although PBL was originally described in 1997 by Delecluse et al. as a human immunodeficiency virus-associated neoplasm typically presenting in the oral cavity, it is now recognized as a PTLD. We describe an unusual and interesting case and to our knowledge the first case of an infant who developed diffuse multifocal cutaneous and systemic PBL shortly after small bowel and liver transplant. We report a case of a 14-month-old female child who received a small bowel and liver transplant from her father. She had excellent graft function with no rejection episodes. Five months post-transplant she developed a sudden gastrointestinal bleed and was noted to have a constantly rising EBV titer despite ongoing maximal antiviral therapy. A patchy erythematous rash was noted on her abdomen that was diagnosed as PBL–PTLD. By the time of this diagnosis, she had developed multiorgan failure unresponsive to therapy.