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.

Paediatric cutaneous lymphomas: a review and comparison with adult counterparts

“…. Reports of paediatric cases of plasmablastic lymphoma are scarce and rare patients were obviously reported by different groups twice . Both types have been reported, including HIV‐positive and ‐negative cases, patients after organ transplantation, lesions confined to the skin at presentation and occurrence in unusual sites (vulva) …”

Orofacial diseases in solid organ and hematopoietic stem cell transplant recipients

“…Gastrointestinal involvement in PTLDs is suggestive of oral PTLD, but definitive diagnosis is by biopsy (Figures 2 and 3). Reports of oral PTLDs are few, with 25 cases located in the literature – more than half in renal transplant recipients (reviewed in: Gonzalez‐Cuyar et al , 2007; Ojha et al , 2008; León et al , 2011; other case reports: Papadaki et al , 2000; Wang et al , 2000; Henry et al , 2008; Apichai et al , 2009). Only two cases have been found among HSCT recipients (Raut et al , 2000; Wang et al , 2000).…”

Long‐term outcomes of living‐related small intestinal transplantation in children: A single‐center experience

“…Our third patient developed a fulminant multifocal cutaneous and systemic plasmablastic lymphoma 5 months after transplantation. Despite treatment with rituximab, she developed multisystem organ failure and died . Our last patient presented PTLD associated with high titers of EBV 40 days after transplantation and was treated with rituximab, methylprednisolone, and cyclophosphamide obtaining favorable results and complete resolution of the disease.…”