Post transplant lymphoproliferative disorder
| Post transplant lymphoproliferative disorder | |
| ICD-O: | M9970/1 |
|---|---|
| DiseasesDB | 34154 |
Template:Post transplant lymphoproliferative disorders Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and Keywords: PTLD
Overview
Historical Perspective
Pathophysiology
Causes
Differentiating Post transplant lymphoproliferative disorder from other Diseases
Epidemiology and Demographics
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings | Other Imaging Findings | Other Diagnostic Studies
Treatment
Medical Therapy | Surgery | Cost-Effectiveness of Therapy | Future or Investigational Therapies
Case Studies
Overview
Post-transplant lymphoproliferative disorder (PTLD) is the name given to a group of B cell lymphomas occurring in immunosuppressed patients following organ transplant.
Incidence and 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, 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.
Resources
Related chapters
References
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