Post transplant lymphoproliferative disorder

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Post transplant lymphoproliferative disorder
ICD-O: M9970/1
DiseasesDB 34154

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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

Case #1


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.

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