Post transplant lymphoproliferative disorder

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: PTLD;

Overview

Post-transplant lymphoproliferative disorder (also known as PTLD) is defined as a B-cell proliferation due to therapeutic immunosuppression after organ transplantation. Patients with post-transplant lymphoproliferative disorder may develop infectious mononucleosis-like lesions or polyclonal polymorphic B-cell hyperplasia. In some cases, B-cells may undergo mutations which will render them malignant, giving rise to a lymphoma. The malignant cell clone can become the dominant proliferating cell type, leading to a group of B cell lymphomas occurring in immunosuppressed patients following organ transplant.

Historical Perspective

  • Post transplant lymphoproliferative disorder was first discovered by Denis Parsons Burkitt, an Irish physician, in 1965.

Classification

  • There is no classification for post transplant lymphoproliferative disorder.

Pathophysiology

  • Post transplant lymphoproliferative disorder arises from germinal center or post-germinal center B cells (B-PTLD), which are normally involved the production of antibodies and durable memory B cells.
  • The pathogenesis of post transplant lymphoproliferative disorder is characterized by the production of interleukin-10.
  • The overexpression of bcl-2 has been associated with the development of post transplant lymphoproliferative disorder.
  • On gross pathology, characteristic findings of post transplant lymphoproliferative disorder, include:
  • No remarkable findings
  • On microscopic histopathological analysis, characteristic findings of post transplant lymphoproliferative disorder, include:
  • Resemblance to large cell lymphomas
  • Large lymphoid cells with a diameter (2x a resting lymphocyte)

Causes

  • The most common causes of post transplant lymphoproliferative disorder is Epstein-Barr virus.

Differentiating Post Transplant Lymphoproliferative Disorder from Other Diseases

  • Post transplant lymphoproliferative disorder must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
  • [Differential dx1]
  • [Differential dx2]
  • [Differential dx3]

Epidemiology and Demographics

  • Post transplant lymphoproliferative disorder is very rare.
  • The prevalence of post transplant lymphoproliferative disorder remains unknown.

Age

  • Post transplant lymphoproliferative disorder is more commonly observed among young patients.

Gender

  • Females are slightly more affected with post transplant lymphoproliferative disorder than men.

Race

  • There is no racial predilection for post transplant lymphoproliferative disorder.

Risk Factors

  • The most common risk factors in the development of post transplant lymphoproliferative disorder is B cell neoplasm associated with Epstein-Barr infection.

Natural History, Complications and Prognosis

  • The majority of patients with post transplant lymphoproliferative disorder remain asymptomatic for [duration/years].
  • Early clinical features include
  • If left untreated, patients with post transplant lymphoproliferative disorder may progress to develop
  • Common complications of post transplant lymphoproliferative disorder, include:
  • Prognosis is generally poor, and the 5-year survival rate of patients with post transplant lymphoproliferative disorder is approximately

Diagnosis

Symptoms

  • Post transplant lymphoproliferative disorder is usually asymptomatic.
  • Symptoms of post transplant lymphoproliferative disorder may include the following:
  • Swelling in the lymph nodes in the neck or underarms

Physical Examination

  • Patients with post transplant lymphoproliferative disorder usually appear pale and malnourished.
  • Physical examination may be remarkable for:
  • Fever
  • Night sweats
  • Persistent fatigue
  • Loss of appetite
  • Nausea
  • Vomiting
  • Unexplained weight loss

Laboratory Findings

  • There are no specific laboratory findings associated with post transplant lymphoproliferative disorder.

Imaging Findings

  • There are no imaging findings associated with post transplant lymphoproliferative disorder.

Treatment

Medical Therapy

  • The medical treatment for post transplant lymphoproliferative disorder, includes: [1]
  • Immunosuppression
  • Antiviral therapy
  • Interferon alpha therapy
  • CD20 antibody therapy
  • Chemotherapy
  • Post-transplant lymphoproliferative disorder may regress spontaneously on reduction or cessation of immunosuppressant medication anti-viral therapy.

Surgery

  • Surgery is not recommended for patients with post transplant lymphoproliferative disorder.

Prevention

  • There are no primary preventive measures available for post transplant lymphoproliferative disorder.

References

  1. BioMed Central. EBV-associated post-transplantation B-cell lymphoproliferative disorder following allogenic stem cell transplantation for acute lymphoblastic leukaemia: tumor regression after reduction of immunosuppression - a case report. https://diagnosticpathology.biomedcentral.com/articles/10.1186/1746-1596-5-21 Accessed on May 23, 2016