Post Transplant Lymphoproliferative Disorders (PTLD)

Post Transplant Post Transplant Lymphoproliferative Lymphoproliferative DisordersDisorders(PTLD)(PTLD)

PTLDPTLDPTLD 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

IncidenceIncidencePTLD 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 Incidence by Type of

Organ TransplantedOrgan TransplantedIntestinal transplant 18%Lung 8%Heart 3%Liver 3%Renal <1%

Clinical PresentationClinical PresentationSome patients are asymptomaticOthers may have non specific

symptoms Fever Malaise Weight loss

May resemble infectious mononucleosisEnlarged tonsilsLymphadenopathyIntestinal Perforation

Systems Potentially Systems Potentially Involved in PTLDInvolved in PTLDCentral Nervous SystemBone MarrowKidneysLiverLungsSmall intestinesSpleen

CharacterizationCharacterizationEarly 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 FactorsRisk FactorsEBV seronegative recipientType of Organ transplantedType of immunosuppressionSimultaneous cytomegalovirus disease

Younger AgeMale

Prevention/Prevention/MonitoringMonitoringEBV Surveillance

◦Monitor EBV-DNA load

EBV vaccine◦Not standard treatment

Anti-viral agents◦Questionable value◦Agents used include Acyclovir & Ganciclovir

Treatment of PTLDTreatment of PTLDReduction of Immunosuppression

therapyAnti-viral agents and IVIG are of

limited valueInterferon alphaRituximabCHOP

◦Doxorubicin, Cyclophosphamide, Vincristine and Prednisone

ConclusionConclusionPrognosis is poor5 year survival rates◦25 to 60%

High mortality rateLack of standard treatment

ReferencesReferencesBakker, 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