EBV Lymphoproliferative disorder
also see: X-linked lymphoproliferative disorder (XLP)

• Reactivation of EBV (Epstein-Barr virus) after bone marrow transplantation can result in EBV-associated lymphoproliferative disease (EBV-LPD).

• EBV is a human herpes virus that infects the majority of individuals by adulthood.

• Primary EBV infection in infants and children is generally asymptomatic, but in adolescence or later life it causes infectious mononucleosis (IM).

• EBV is aetiologically associated

  • Burkitt lymphoma

  • nasopharyngeal carcinoma (NPC)

  • some forms of Hodgkin disease

  • gastric cancers.

• EBV has also been implicated as causative agent in a wide range of lymphoproliferative disorders in immunocompromised patients, such as transplant recipients and those with inherited or acquired immunodeficiencies

• The host response to EBV infection is still not understood in detail. EBV infection of B-lymphocytes results in their proliferation, which, in normal individuals, is controlled by an antigen-specific MHC-restricted cytotoxic T-cell response.

Rituximab, alone or in addition to other therapies, promises a profound change in the landscape with regard to the treatment and perhaps the prevention of posttransplant lymphoproliferative disease. New approaches to adoptive cellular immunotherapy, including use of EBV-specific products from unrelated donors, nonspecifically activated autologous products, and genetically engineered T cells, are all being explored.

Uncontrolled expansion of donor-derived Epstein-Barr virus (EBV)-infected B cells has become a significant problem in recipients of allogeneic hematopoietic stem cell transplantations.

Major risk factors for the early development of post-transplantation lymphoproliferative disease include

• the use of unrelated or HLA-mismatched related donors

• selective T-cell depletion of donor marrow

• use of antithymocyte globulin or monoclonal anti-T-cell antibodies for the prophylaxis and treatment of acute graft-versus-host disease.

Notes taken during BMT rotation at CHLA 2003