Post Transplant Lymphoproliferative Disease (PTLD) - Pearls

PTLD is almost always a B-cell neoplasm, and is associate with EBV in >80% of cases
pediatric patients are at higher risk, primarily b/c most of them are EBV-negative at time of transplant
incidence after solid organ tx is highest in lung tx>>heart>>kidney
risk is most related to the degree of immunosuppression and the pt's pre-transplant EBV status
PTLD consists of a wide variety of lymphoproliferative diseases. The WHO classifies them into 3 categories: early lesions (plasmocytic hyperplasia, or mononucleosis-like lesions), monomorphic PTLD: B-cell neoplasms (diffuse large B-cell lymphoma, Burkitt lymphoma, plasma cell myeloma etc.) and polymorphic PTLD (having multiple cell types, often related to primary EBV infxn)
Epidemiology:

incidence after solid organ tx is highest in lung tx>>heart>>kidney
risk is most related to the degree of immunosuppression and the pt's pre-transplant EBV status
with respect to immunosuppressive drugs, tacrolimus is associated with higher rates of PTLD than cyclosporine or cellcept
pediatric patients are at higher risk, primarily b/c most of them are EBV-negative at time of transplant

Clinical presentation is often insidious, like an infectious mononucleosis syndrome, also can cause cytopenias and a monoclonal B-cell proliferation. Over 50% of patients present with an extranodal mass (can be in the GI tract, lungs, skin, CNS); up to 25% can present with infiltration into the graft organ
Diagnosis requires a high level of suspicion as the disorder often presents subtly and extranodal; radiographic evidence of a mass and elevated serum markers (such as LDH) are suggestive of PTLD in a transplant pt—PET CT can be helpful in making the diagnosis
PTLD consists of a wide variety of lymphoproliferative diseases. The WHO classifies them into 3 categories: early lesions (plasmocytic hyperplasia, or mononucleosis-like lesions), monomorphic PTLD: B-cell neoplasms (diffuse large B-cell lymphoma, Burkitt lymphoma, plasma cell myeloma etc.) and polymorphic PTLD (having multiple cell types, often related to primary EBV infxn)

(Victoria Kelly MD, 11/2/10, 6/9/11)