Posttransplant lymphoproliferative disease
From IDWiki
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
- B-cell neoplasms
- 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 rituximab