Post-transplant lymphoproliferative disorder
| Post-transplant lymphoproliferative disorder | |
|---|---|
| Classification and external resources | |
| ICD-9 | 238.77 |
| ICD-O: | M9970/1 |
| DiseasesDB | 34154 |
| eMedicine | ped/2851 |
Post-transplant lymphoproliferative disorder (PTLD) is the name given to a B-cell proliferation due to therapeutic immunosuppression after organ transplantation. These patients may develop infectious mononucleosis-like lesions or polyclonal polymorphic B-cell hyperplasia. Some of these B-cells may undergo mutations which will render them malignant, giving rise to a lymphoma.[citation needed]
In some patients, the malignant cell clone can become the dominant proliferating cell type, leading to frank lymphoma, a group of B cell lymphomas occurring in immunosuppressed patients following organ transplant.
Causes [edit]
The disease is an uncontrolled proliferation of B cell lymphocytes following infection with Epstein-Barr virus.[1] 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, ALG and OKT3.
Polyclonal PTLD may form tumor masses and present with symptoms due to a mass effect, e.g. symptoms of bowel obstruction. Monoclonal forms of PTLD tend to form a disseminated malignant lymphoma.
Treatment [edit]
PTLD may spontaneously regress on reduction or cessation of immunosuppressant medication,[2] and can also be treated with addition of anti-viral therapy. In some cases it will progress to non-Hodgkin's lymphoma and may be fatal.
References [edit]
- ^ Gottschalk S, Rooney CM, Heslop HE (2005). "Post-transplant lymphoproliferative disorders". Annu. Rev. Med. 56 (1): 29–44. doi:10.1146/annurev.med.56.082103.104727. PMID 15660500.
- ^ "Hematopathology".
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