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 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

• The prevalence of post transplant lymphoproliferative disorder is approximately [number or range] per 100,000 individuals worldwide.

Age

• Post transplant lymphoproliferative disorder is more commonly observed among patients aged [age range] years old.
• Post transplant lymphoproliferative disorder is more commonly observed among [elderly patients/young patients/children].

Gender

• Post transplant lymphoproliferative disorder affects men and women equally.

Race

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

Risk Factors

• Common risk factors in the development of post transplant lymphoproliferative disorder are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The majority of patients with post transplant lymphoproliferative disorder remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, patients with post transplant lymphoproliferative disorder may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of post transplant lymphoproliferative disorder include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/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 [general appearance].
• 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:

• 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.