| 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 group of B cell lymphomas occurring in immunosuppressed patients following organ transplant.
Contents |
Incidence/prevalence
It is an uncommon condition occurring in 0.2% of patients within one year of transplant, with an annual incidence of 0.04% thereafter. The risk of developing the disease is higher in children and recipients of small bowel transplants.
Causes
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
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
- ^ Gottschalk S, Rooney CM, Heslop HE (2005). "Post-transplant lymphoproliferative disorders". Annu. Rev. Med. 56: 29–44. doi:. PMID 15660500. http://arjournals.annualreviews.org/doi/abs/10.1146/annurev.med.56.082103.104727?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dncbi.nlm.nih.gov.
- ^ "Hematopathology". http://library.med.utah.edu/WebPath/HEMEHTML/HEME099.html.
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