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Transplantationsassoziierte lymphoproliferative Erkrankungen (PTLD) bei Kindern

Pathogenese, klinische Symptome, Diagnostik und Therapie

Post-transplant lymphoproliferative diseases in children

Pathogenesis, clinical symptoms, diagnostics and treatment

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Zeitschrift für Herz-,Thorax- und Gefäßchirurgie Aims and scope

Zusammenfassung

Die Transplantationsassoziierte lymphoproliferative Erkrankung („post-transplant lymphoproliferative disorder“, PTLD) ist eine nach Organtransplantation auftretende seltene, schwere Komplikationen der immunsuppressiven Therapie. Die Erkrankungen sind häufig assoziiert mit einer Infektion durch oder Reaktivierung von Epstein-Barr-Virus und beruhen auf einer gestörten virusspezifischen Immunkompetenz. Pro Jahr erkranken in Deutschland etwa 15 bis 20 Kinder an PTLD. An der Behandlung sind pädiatrische Transplantationsmediziner und pädiatrische Onkologen interdisziplinär beteiligt. Die Symptome der PTLD sind vielfältig und die Diagnosestellungen häufig schwierig. Histologisch wird die PTLD in 6 verschiedene Untergruppen aufgeteilt. Die Therapie sieht gemäß dem Ansprechen stratifizierte Maßnahmen in Form von Reduktion der immunsuppressiven Therapie, Gabe des Anti-CD20-Antikörpers Rituximab und Anwendung eines an die Immunsuppression adaptierten Chemotherapieschemas vor. Die Patienten werden im Ped-PTLD-Register mit Sitz in Hannover erfasst; dieses bietet Hilfestellungen in der Diagnostik und Beratung bei der Behandlung an.

Abstract

Post-transplantation lymphoproliferative disorders (PTLD) are rare but severe complications of immunosuppressive therapy following solid organ transplantation. The diseases are often associated with prior infection with or reactivation of Epstein-Barr virus and are based on a disturbed virus-specific immune response. In Germany, approximately 15–20 children develop PTLD per year. Interdisciplinary treatment involves pediatric transplantation specialists and pediatric oncologists. The symptoms of PTLD are manifold and the diagnostics are often difficult. Based on histology, PTLD is divided into six subgroups. Stratified treatment involves reduction of immunosuppressive therapy, administration of the anti-CD20 antibody rituximab and use of an immunosuppression adapted chemotherapy regimen. Patients are registered in the Ped-PTLD registry (Hannover Medical School, Germany), which offers assistance in the diagnostics and counselling for the treatment.

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Authors and Affiliations

  1. Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    Rebecca E. Schultze-Florey &  Britta Maecker-Kolhoff

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  1. Rebecca E. Schultze-Florey
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  2. Britta Maecker-Kolhoff
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Correspondence to Rebecca E. Schultze-Florey.

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R. Schultze-Florey und B. Maecker-Kolhoff geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Schultze-Florey, R.E., Maecker-Kolhoff, B. Transplantationsassoziierte lymphoproliferative Erkrankungen (PTLD) bei Kindern. Z Herz- Thorax- Gefäßchir 32, 502–509 (2018). https://doi.org/10.1007/s00398-018-0272-4

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