Authors: Gyula Végső Melinda Hajdu Anna Sebestyén
Publish Date: 2010/12/31
Volume: 17, Issue: 3, Pages: 443-454
Abstract
Posttransplant lymphoproliferative disorder PTLD is a heterogeneous disease group of benign and malignant entities The new World Health Organisation classification introduced in 2008 distinguishes early lesions polymorphic monomorphic and classical Hodgkin lymphomatype PTLD Based on the time of appearance early and late forms can be identifiedPTLDs are the second most frequent posttransplantation tumors in adulthood and the most frequent ones in childhood The incidence varies with the transplanted organ—from 1–2 following kidney transplantation to as high as 10 following thoracic organ transplantation—due to different intensities in immunosuppression Immunocompromised state and EpsteinBarr virus EBV infection are the two major risk factorsIn Europe and the US approximately 85 of PTLDs are of Bcell origin and the majority are EBVassociated Symptoms are often unspecific extranodal organ manifestations and central nervous system involvement is common Early lesions respond well to a decrease in immunosuppression Malignant entities are treated with rituximab chemotherapy radiotherapy and surgical therapy Adoptive Tcell transfer represents a promising therapeutic approach The prognosis is favorable in early PTLD and poor in late PTLD Fiveyear survival is 30 for highgrade lymphomas The prognosis of EBVnegative lymphomas is worseLowering the risk of PTLD may be achieved by low dose maintenance immunosuppression immunosuppressive drugs inhibiting cell proliferation and special immunotherapy eg interleukin2 inhibitors Early detection is especially important for high risk—eg EBVnegative—patients where the appearance of EBVDNA and the increase in its titer may help
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