View source for Posttransplant lymphoproliferative disease

==Background==
* Hematologic neosplasia caused by [[Epstein-Barr virus]] that follows [[solid organ transplantation]] or [[hematopoietic stem cell transplantation]]

===Pathophysiology===
* 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

==Management==
* Treated with [[Is treated by::rituximab]]