Posttransplant lymphoproliferative disease

From IDWiki

Background

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