A case of fulminant post‐transplant lymphoproliferative disorder and septicemia

Abstract

The fulminant form of post-transplant lymphoproliferative disorder (PTLD) is very uncommon and occurs in approximately 1% of PTLD patients. Approximately 85% of these lesions are of B-cell origin, and most of them are associated with Epstein-Barr virus infection. Fulminant PTLD is frequently associated with a systemic inflammatory response syndrome, and may be difficult to differentiate from septicemia. We describe the case of a 59-yr-old man who suffered from prolonged septicemia in the immediate post-transplant period, and presented again four months after cardiac transplantation with fever, painful liver edge and gastrointestinal bleeding. The diagnosis of fulminant PTLD with advanced multiorgan infiltration by a diffuse large-cell lymphoma of B-cell phenotype was made. During treatment with rituximab, the patient died from Enterococcus faecium septicemia. The sequence of septicemia, PTLD and, finally again, septicemia is an unusual challenge and urges for an aggressive diagnostic approach, where markers like procalcitonin may aid in the discrimination of fulminant PTLD from septicemia.

Post‐transplant lymphoproliferative disease in children, adolescents, and young adults

“…Involvement of the central nervous system is rare although when present it can manifest with acute neurologic changes 44 . Rarely but significantly, patients can present in a hyperinflammatory state with multiorgan failure known as fulminant PTLD which can be difficult to differentiate from sepsis 45 …”

Posttransplant Lymphoproliferative Disorders (PTLDs)

“…Following liver transplantation, PTLD may develop in the liver, but overall this is a rare complication (Barkholt et al 1991;Mulvihill et al 1994;Pickhardt and Siegel 1999;Nuckols et al 2000;Wu et al 2001;Jain et al 2002;Gouya et al 2006;Avolio et al 2007;Ulu et al 2007). Hepatic PTLD in a grafted liver may be of donor origin (Spiro et al 1993).…”

Posttransplant Lymphoproliferative Disease Presenting as an Extracranial Mass

“…Identifying patients with PTLD remains challenging not least because of the variety of initial clinical manifestations. These range from nonspecific presentations such as fever, weight loss, and night sweats to lymphoma-like masses in native organs and even overt sepsis [ 7 , 8 ]. Soft tissue manifestations of PTLD at extracranial sites are rare and if not recognized in a timely manner can result in delay of diagnosis and treatment.…”