Post-transplant lymphoproliferative disease

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As a post-transplant lymphoproliferative disorder or post-transplant lymphoproliferative disease , according to the English term post-transplant lymphoproliferative disorder abbreviated as PTLD , various lymphoma-like disease called after an organ transplant or a stem cell transplant may occur. It is a group of histologically and molecular-biological heterogeneous diseases, the spectrum of which ranges from benign proliferation of T and B lymphocytes to malignant lymphomas . After Kaposi's sarcoma and other skin cancers, PTLD is the second most common tumor-like disease after an organ transplant.

distribution

From an epidemiological point of view, up to ten percent of all organ transplant patients develop PTLD. The individual risk depends on the transplanted organ , the immunosuppressive therapy, the presence of an infection with the Epstein-Barr virus (EBV) at the time of the transplant and the age of the patient concerned. With regard to immunosuppression, the overall burden of the dose of the medication and the duration of the treatment is decisive; differences between different medications or treatment regimens have not yet been shown. Children and patients who were EBV-negative before the transplant have a significantly higher risk of developing PTLD after the transplant.

Cause and development of the disease

In most cases, the cause of PTLD is the combination of an EBV infection and the immunosuppressive treatment that is necessary after an organ transplant to suppress the rejection of the foreign organ. In the case of PTLD within the first year after the transplantation, an association with the Epstein-Barr virus can be demonstrated in around 90 percent of cases, while in up to 45 percent of the later occurrences no evidence of EBV infection of the affected cells is possible. In Europe and the USA , around 85 percent of all PTLD cases can be traced back to B lymphocytes and around 15 percent to T lymphocytes; in some other regions, the proportion of T-cell-associated diseases is higher.

Clinical manifestations

The clinical picture of a PTLD depends on the localization. Depending on the severity of the disease, the symptoms are comparable to those of Pfeiffer glandular fever , Hodgkin's disease or the various forms of non-Hodgkin's lymphoma . A manifestation similar to infectious mononucleosis occurs especially in childhood. The symptoms are mostly unspecific and can include fever , fatigue, night sweats and weight loss as well as signs of tonsillitis , sinusitis or otitis media . Most patients have at least one manifest tumor, which in most cases lies outside the lymph nodes and can lead to symptoms such as pain, bleeding or, if localized in the central nervous system , neurological deficits. Depending on the transplanted organ, this itself can be affected.

Investigation methods

The diagnosis of PTLD by means of a biopsy by histological methods as well as the detection of EBV-specific DNA by polymerase chain reaction (PCR). The pathological examination is especially important for the determination of the clonality and the degree of malignancy , the PCR-based determination of the viral load is also suitable for follow-up monitoring.

Treatment and prospect of recovery

After weakening or discontinuation of immunosuppressive therapy, a regression of PTLD occurs in many cases. The treatment is therefore carried out on the one hand by adjusting the dosage of the immunosuppressive drugs and on the other hand, as with other lymphoma diseases, depending on the severity of the PTLD, by surgical removal of solid tumors, chemotherapy and radiation therapy and various immune stimulation methods . The therapeutic antibody rituximab plays an important role in drug treatment . Both for therapy and for prophylaxis are beyond antivirals used.

The course of the disease depends on the individual characteristics of the PTLD, in particular the degree of malignancy and location in the body, as well as the response to treatment. If PTLD occurs more than two years after organ transplantation, reducing immunosuppressive treatment is usually less effective than if PTLD occurs within the first two years. The published data on lethality are sometimes over 50 percent.

Individual evidence

  1. ↑ OM Martinez, FR de Gruijl: Molecular and Immunologic Mechanisms of Cancer Pathogenesis in Solid Organ Transplant Recipients. In: American Journal of Transplantation . 8/2008, pp. 2205-2211
  2. ↑ a b c A.L. Taylor, R. Marcus, J. Andrew Bradley: Post-transplant lymphoproliferative disorders (PTLD) after solid organ transplantation. In: Critical Reviews in Oncology Hematology . 56 (1 )/2005. Elsevier, pp. 155-167, ISSN  1040-8428
  3. ↑ a b c R. Dolcetti: B lymphocytes and Epstein-Barr virus: The lesson of post-transplant lymphoproliferative disorders. In: Autoimmunity Reviews . 7 (2) / 2007. Elsevier, pp. 96-101, ISSN  1568-9972
  4. ↑ a b c W.H. Lim, GR Russ, PT Coates: Review of Epstein-Barr virus and post-transplant lymphoproliferative disorder post-solid organ transplantation. In: Nephrology. 11 (4) / 2006. Asian Pacific Society of Nephrology, pp. 355-366, ISSN  1320-5358
  5. ↑ J. Svoboda, R. Kotloff, DE Tsai: Management of patients with post-transplant lymphoproliferative disorder: the role of rituximab. In: Transplant International . 19: pp. 259-269, 2006 ISSN  0934-0874
  6. ↑ MJ Everly, RD Bloom, DE Tsai, J. Trofe: Posttransplant lymphoproliferative disorder. In: The Annals of Pharmacotherapy . 41 (11 )/2007. Highwire Press, pp. 1850-1856, ISSN  1060-0280

literature

  • Anna L. Taylor, Robert Marcus, J. Andrew Bradley: Post-transplant lymphoproliferative disorders (PTLD) after solid organ transplantation. In: Critical Reviews in Oncology / Hematology. 56 (1 )/2005. Elsevier, pp. 155-167, ISSN  1040-8428
  • Stephen Gottschalk, Cliona M. Rooney, Helen E. Heslop: Post-Transplant Lymphoproliferative Disorders. In: Annual Review of Medicine . 56/2005. Annual Reviews, pp. 29-44, ISSN  0066-4219