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Post Transplant Lymphoproliferative Disorders (PTLD). PTLD. PTLD is a group of lymphoproliferative disorders that range from hyperplasias to aggressive non-Hodgkin’s lymphoma PTLD is seen after both solid organ and stem cell transplant 90% of PTLDs are Ebstein-Barr Virus (EBV) positive.
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PTLD • PTLD is a group of lymphoproliferative disorders that range from hyperplasias to aggressive non-Hodgkin’s lymphoma • PTLD is seen after both solid organ and stem cell transplant • 90% of PTLDs are Ebstein-Barr Virus (EBV) positive
Incidence • PTLD occurs in 1% to 25% of solid organ transplant patients • PTLD occurs in < 2% of stem cell transplant patients • Incidence is 4 times higher in pediatric transplant patients versus adults
Incidence by Type of Organ Transplanted • Intestinal transplant 18% • Lung 8% • Heart 3% • Liver 3% • Renal <1%
Clinical Presentation • Some patients are asymptomatic • Others may have non specific symptoms • Fever • Malaise • Weight loss • May resemble infectious mononucleosis • Enlarged tonsils • Lymphadenopathy • Intestinal Perforation
Systems Potentially Involved in PTLD • Central Nervous System • Bone Marrow • Kidneys • Liver • Lungs • Small intestines • Spleen
Characterization • Early PTLD presents within one year following transplantation • 60% develop within one year • Late PTLD occurs more than one year post transplant • 30% develop within 1 to 5 years • 10% develop after 5 years
Risk Factors • EBV seronegative recipient • Type of Organ transplanted • Type of immunosuppression • Simultaneous cytomegalovirus disease • Younger Age • Male
Prevention/Monitoring • EBV Surveillance • Monitor EBV-DNA load • EBV vaccine • Not standard treatment • Anti-viral agents • Questionable value • Agents used include Acyclovir & Ganciclovir
Treatment of PTLD • Reduction of Immunosuppression therapy • Anti-viral agents and IVIG are of limited value • Interferon alpha • Rituximab • CHOP • Doxorubicin, Cyclophosphamide, Vincristine and Prednisone
Conclusion • Prognosis is poor • 5 year survival rates • 25 to 60% • High mortality rate • Lack of standard treatment
References Bakker, N.A., van Imhoff, G.W., Verschuuren, E.A.M., & van Son, W. (2006). Presentation and early detection of post transplant lymphoproliferative disorder after solid organ transplantation.Transplant International, 20, 207-218 Everly, M.J., Bloom, R.D., Tsai, D.E. & Trofe, J. (2007). Posttransplant Lymphoproliferative Disorder. The Annals of Pharmacotherapy, 41, 1850-1858 Faye, A. & Vilmer, E. (2005). Post-Transplant Lymphoproliferative Disorder. Pediatric Drugs, 7, 1, 55-65 Gottschalk, S., Rooney, C.M., & Heslop, H.E. (2005). Post-Transplant Lymphoproliferative Disorders, 56, 29-44 Lee, J.J., Lam, M.S.H., & Rosenberg, A. (2007). Role of Chemotherapy and Rituximab for Treatment of Posttransplant Lymphoproliferative Disorder in Solid Organ Transplantation. The Annals of Pharmacotherapy, 41, 1648-1659 Shroff, R. & Rees, L. (2003). The post-transplant lymphoproliferative disorder – a literature review. Pediatric Nephrology, 19, 369 -377