Post-transplant lymphoproliferative disorder
- Post-transplant lymphoproliferative disorder
Infobox_Disease
Name = PAGENAME
Caption =
DiseasesDB = 34154
ICD10 =
ICD9 =
ICDO = M9970/1
OMIM =
MedlinePlus =
eMedicineSubj = ped
eMedicineTopic = 2851
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Post-transplant lymphoproliferative disorder (PTLD) is the name given to a group of B cell lymphomas occurring in immunosuppressed patients following organ transplant.
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 heart transplants.
Causes
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, 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,[cite web |url=http://library.med.utah.edu/WebPath/HEMEHTML/HEME099.html |title=Hematopathology |format= |work= |accessdate=] 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
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