Posttransplant Lymphoproliferative Disorder B27.0

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 31.03.2025

Synonym(s)

Lymphoproliferative disease after transplantation; Post-Graft Lymphoproliferative Disorder; Posttransplantation Lymphoproliferative Disorders (e); Posttransplant Lymphoproliferative Disorders; PTLD

Definition
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Group of histologically and molecularly heterogeneous, lymphoproliferative diseases that can occur after allogeneic hematopoietic stem cell transplantation or solid organ transplantation. The spectrum ranges from benign T- and B-cell proliferations to malignant lymphomas.

After Kaposi's sarcoma and other melanocytic and non-melanocytic malignant skin tumors, PTLD is the second most common tumor-like disease after organ transplantation and therefore also represents a relevant clinical-diagnostic challenge for dermatologists.

Classification
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A distinction is made according to the time of occurrence:

Early PTDL within the first 12 months after transplantation (EBV positive)
Late PTDL 5-10 years after transplantation (EBV negative)

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Occurrence/Epidemiology
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In Europe and the USA, about 85% of all PTLD cases are due to B-lymphocyte proliferation and about 15% to T-cell proliferation. Depending on the transplanted organ and the age of the patient, the risk of developing PTLD is between 0.5% and 20% of transplanted patients. Children as well as patients who were EBV-negative before the transplantation have a significantly higher risk of developing PTLD after the transplantation.

Etiopathogenesis
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The cause of PTLD is thought to be the coincidence of EBV infection and the underlying immunosuppressive treatment. In immunocompromised patients, increased viral replication and a higher number of latent EBV-infected B-lymphocytes in peripheral blood are found. In these cells EBV acts as a continuous proliferation stimulus.

Clinical features
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Cutaneous manifestations of lymphoproliferative diseases (PTLD) after transplantation are rare. The median interval between the first transplant and diagnosis was 85 months in a larger review (Seçkin D et al. 2013).

The cutaneous manifestations were classified as follows:

primary cutaneous T-cell lymphoma (CTCL) (70% of cases)
primary cutaneous B-cell lymphomas (30% of cases).

Mycosis fungoides (MF) was the most common (50%) CTCL subtype. About 90 )of the cutaneous B-cell PTLD cases were classified as EBV-associated B-cell lymphoproliferations (including one plasmablastic lymphoma and one lymphomatoid granulomatosis) and one as diffuse large B-cell lymphoma.

Prognosis: Approximately 50% of patients died after a median follow-up of 19.5 months Median survival for all patients with CTCL (93 months) and cutaneous B-cell PTLD (112 months). The survival rates for MF were higher than those for CD30(+)-LPD.

The prognosis of primary cutaneous CD30(+) LPD after transplantation is therefore worse than that of mycosis fungoides after transplantation and that of comparable lymphoma entities with immunocompetence (Seçkin D et al. 2013).

Diagnosis
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Histological examination; detection of EBV-specific DNA by PCR.

Therapy
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In many cases, PTLD regression occurs after the immunosuppressive therapy has been weakened or discontinued. The treatment is carried out by adjusting the dosage of the immunosuppressive drugs; the procedure remains the same as for other lymphoma diseases.

Progression/forecast
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The course of the disease depends on the individual characteristics of the PTLD, in particular on the degree of malignancy. The mortality rate is sometimes > 50%.

Note(s)
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The staging of PTLD is analogous to the Ann Arbor classification for non-Hodgkin's lymphoma.

Lymphoproliferative diseases have also been described after long-term treatment of rheumatoid diseases with methotrexate (Fujita Y et al. 2025)

Literature
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Aida N et al. (2019) A Case of Epstein-Barr Virus-Associated Leiomyosarcoma Concurrently With
Posttransplant Lymphoproliferative Disorders After Renal Transplantation.
Clin Med Insights Case Rep 12:1179547619867330.
Fujita Y et al. (2025) Cutaneous methotrexate-related lymphoproliferative disorder mimicking nodular lymphangitis. J Dtsch Dermatol Ges. 23:218-220.
Seçkin D et al. (2013) Primary cutaneous posttransplant lymphoproliferative disorders in solid organ transplant recipients: a multicenter European case series. Am J Transplant 13:2146-2153.
Trappe, R et al. (2006) Pathogenic, Clinical, Diagnostic and Therapeutic Aspects of Posttransplantation Lymphoproliferative Disorders. Dtsch Arztebl 103: A-3259 / B-2836 / C-2718