Uncontrolled proliferation of EBV-infected B-cells
Half occur with reactivation of latent infection and half after primary infection
Epidemiology
Complicates about 1% of SCT; 1-2.5% of renal, liver, and heart transplants; 6% of lung transplants; 10% of intestinal transplants; and up to one third of multivisceral transplants
Higher risk in T-cell-depleted stem cell transplants or umblical cord transplants
Clinical Manifestations
Classically presented with a mononucleosis-like syndrome, with fever and lymphadenopathy
Can involve lymph nodes, spleen, liver, bone marrow, kidney, lung, CNS, and intestine
Highest risk in HSCT is within the first five months, but in SOT can be prolonged depending on the duration and intensity of immunosuppression
In HSCT, late PTLD is more common in older patients and usually presents as extranodal mass lesions involving CNS, head and neck, or bowels
Similar to non-Hodgkin lymphoma, with fewer constitutional symptoms
High mortality >70%
Can be EBV negative
Categories of PTLD
Early lesions
Plasmacytic hyperplasia
Infectious mononucleosis-like lesion
Polymorphic PTLD
Monomorphic PTLD
B-cell neoplasms
Diffuse large B-cell lymphoma
Burkitt lymphoma
Plasma cell myeloma
Plasmacytoma-like lesion
T-cell neoplasms
Peripheral T-cell lymphoma
Hepatosplenic T-cell lymphoma
Others
Class Hodgkin lymphoma-like PTLD
Prevention
High-risk hematopoietic transplant recipients are monitored with EBV viral load weekly until at least 3 months posttransplantation
If viral load elevated (which can precede disease by 3 weeks), it is treated either with decreasing immunosuppression or with rituximab, or with anti-EBV T-cell infusions
