Posttransplant lymphoproliferative diseaseB27.0
Synonym(s)
Lymphoproliferative disease after transplantation; Post-Graft Lymphoproliferative Disorder; Posttransplantation Lymphoproliferative Disorders (e); Posttransplant Lymphoproliferative Disorders; PTLD
DefinitionThis section has been translated automatically.
Group of histologically and molecularly heterogeneous lymphoproliferative diseases that may occur after allogeneic hematopoietic stem cell transplantation or after transplantation of solid organs. 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 tumours, PTLD is the second most common tumour-like disease after organ transplantation and thus also represents a relevant disease for dermatologists.
ClassificationThis section has been translated automatically.
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)
Occurrence/EpidemiologyThis section has been translated automatically.
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.
EtiopathogenesisThis section has been translated automatically.
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 featuresThis section has been translated automatically.
The clinical picture of a PTLD depends on the localization and the lymphoproliferative clone. Morphologically, the different forms of non-Hodgkin's lymphoma can appear in adulthood, both B-cell and T-cell lymphomas (T-cell PTLD).
DiagnosisThis section has been translated automatically.
Histological examination; detection of EBV-specific DNA by PCR.
TherapyThis section has been translated automatically.
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/forecastThis section has been translated automatically.
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)This section has been translated automatically.
The stage classification of PTLD is similar to the Ann-Arbor classification for non-Hodgkin's lymphomas.
LiteratureThis section has been translated automatically.
- Aida N et al (2019) A Case of Epstein-Barr Virus-Associated Leiomyosarcoma Concurrently WithPosttransplant
Lymphoproliferative Disorders After Renal Transplantation.
Clin Med Insights Case Rep 12:1179547619867330. - Trappe, R et al (2006) Pathogenic, Clinical, Diagnostic and Therapeutic Aspects of Posttransplantation Lymphoproliferative Disorders. Dtsch Arztebl 103: A-3259 / B-2836 / C-2718