Post-transplant lymphoproliferative disorder
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| Post-transplant lymphoproliferative disorder Classification and external resources | |
| ICD-O: | M9970/1 |
|---|---|
| DiseasesDB | 34154 |
| eMedicine | ped/2851 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Phone:617-525-6884
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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/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.
Organ transplantation | |
|---|---|
| Types | Allograft · Alloplant · Allotransplantation · Autotransplantation · Xenotransplantation |
| Organs and tissues | Bone · Bone marrow · Corneal · Face · Hand · Heart · Heart-lung · Kidney · Liver · Lung · Pancreas · Penis · Skin · Spleen · Uterus |
| Related topics | Biomedical tissue · Cellular memory · Edmonton protocol · Eye bank · Graft-versus-host disease · Immunosuppressive drugs · Islet cell transplantation · Implants · Living donor liver transplantation · Lung allocation score · Machine perfusion · Medical grafting · Non-heart beating donation · Organ donation · Post-transplant lymphoproliferative disorder · Repugnant market · Total body irradiation · Transplant rejection |
| Organizations | Halachic Organ Donor Society · Human Tissue Authority · National Marrow Donor Program · United Network for Organ Sharing |
| People | Christiaan Barnard · Michael Woodruff · Alexis Carrel · Norman Shumway · Jean-Michel Dubernard · List of notable organ transplant donors and recipients |
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
