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It is an uncommon condition occurring in 0.2% of patients within 1 year of transplant, with an annual incidence of 0.04% thereafter. The risk of developing the disease is higher in children and recipients of heart transplants.
The disease is an uncontrolled proliferation of B cell lymphocytes following infection with Epstein-Barr virus. Production of an interleukin-10, an endogenous anti-T cell cytokine, has also been implicated.
In immunocompetent patients, Epstein-Barr virus causes infectious mononucleosis, characterised by a proliferation of B-lymphocytes which is controlled by Suppressor T cells .
However, calcineurin inhibitors ( tacrolimus and cyclosporine) used as immunosuppressants in organ transplantation inhibit T cell function, and can prevent the control of the B cell proliferation.
Depletion of T cells by use of anti-T cell antibodies in the prevention or treatment of transplant rejection further increases the risk of developing post-transplant lymphoproliferative disorder. Such antibodies include ATG, ALGAnti-lymphocyte globulin (ALG) is an infusion of horse-derived antibodies against human T cells which is used in the treatment of acute rejection in organ transplantation, especially in kidney transplants. It is less commonly used than the similar anti-th and OKT3 .
Polyclonal PTLD may form tumour masses and present with symptoms due to a mass effect, e.g. symptoms of bowel obstructionBowel obstruction can occur at any level in the digestive tract, and is a medical emergency. Although many cases are not treated surgically, it is a surgical problem. Causes Small bowel obstruction Causes of small bowel obstruction include: Adhesions from. Monoclonal forms of PTLD tend to form a disseminated maligant lymphoma.
PTLD may spontaneously regress on reduction or cessation of immunosuppressant medication, and can also be treated with addition of anti-viral therapy. In some cases it will progress to non-Hodgkin's lymphomaNon-Hodgkin's lymphoma is a type of cancer. Lymphoma is a general term for cancers that develop in the lymphatic system. Hodgkin's disease is one type of lymphoma. All other lymphomas are grouped together and are called non-Hodgkin's lymphoma. Lymphomas a and may be fatal.