CNS or Bone Marrow Involvement As Risk Factors for Poor Survival in Post-Transplantation Lymphoproliferative Disorders in Children After Solid Organ Transplantation

Abstract

Stage IV disease is an independent risk factor for poor survival in pediatric PTLD patients. Prospective multicenter trials are needed to delineate additional risk factors and to assess treatment approaches for pediatric PTLD.

Post Transplant Lymphoproliferative Disorders After Liver Transplantation

“…tract (Leblond V et al, 1995). The CNS is involved in up to 30% of cases of PTLD and in many of these the disease is confined to the CNS (Maecker B et al, 2007;Penn I & Porat G, 1995). In this respect, PTLD contrasts with NHL in the general population where only around 1% of cases shows isolated CNS involvement.…”

“…More rarely, solitary lesions were seen in the lung, tonsils and central nervous system (CNS). In particular, CNS involvement, especially in paediatric patients seems to be a risk factor for poor prognosis (Maecker B et al, 2007). In patients presenting with multiple lesions, the lymph nodes and liver were most commonly affected.…”

Posttransplant Lymphoproliferative Disorders Following Solid Organ Transplantation

“…(Faull et al, 2005;Webber et al, 2006).More recent data suggest that the median time is later, at approximately 3 years after SOT. ( Evens et al, 2010;Ghobrial et al, 2010;Knight et al, 2009;Maecker et al, 2007) This is partly due to an increase in the diagnosis of EBV-unrelated disease. Multiple reports have shown that EBV-unrelated PTLD occurs later after SOT, most commonly after 3 to 5 years.…”

“… Recurrent chromosomal abnormalities have been reported in some series of B-cell PTLD and may portend a worse prognosis. (Chadburn et al, 1997;Djorkic et al, 2006;Maecker et al, 2007;Swerdlow et al, 2008).…”

“…(Dotti et al, 2002;Leblond et al, 1998;Nelson et al, 2000;Taylor et al, 2005) Recent data shows an improved response to treatment, which could be explained both by improvements in treatment strategies of the tumors and by improved supportive care. (Elstrom et al, 2006;Evens et al, 2010;Knight et al, 2009;Maecker et al, 2007) …”