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 rituximab