Turk J Hematol. 2014; 31(1): 79-83 | DOI: 10.4274/Tjh.2012.0010

Epstein-Barr Virus-Negative Post-Transplant Lymphoproliferative Diseases: Three Distinct Cases from a Single Center

Şule Mine Bakanay¹, Gülşah Kaygusuz², Pervin Topçuoğlu¹, Şule Şengül³, Timur Tunçalı⁴, Kenan Keven³, Işınsu Kuzu², Akın Uysal¹, Mutlu Arat^1
1Ankara University School Of Medical, Department Of Hematology, Ankara, Turkey
²Ankara University School Of Medical, Department Of Pathology, Ankara, Turkey
³Ankara University School Of Medical, Department Of Nephrology, Ankara, Turkey
⁴Ankara University School Of Medical, Department Of Medical Genetics, Ankara, Turkey

Three cases of Epstein-Barr virus (EBV)-negative post-transplant lymphoproliferative disease that occurred 6 to 8 years after renal transplantation are reported. The patients respectively had gastric mucosa-associated lymphoid tissue lymphoma, gastric diffuse large B-cell lymphoma, and atypical Burkitt lymphoma. Absence of EBV in the tissue samples was demonstrated by both in situ hybridization for EBV early RNA and polymerase chain reaction for EBV DNA. Patients were treated with reduction in immunosuppression and combined chemotherapy plus an anti-CD20 monoclonal antibody, rituximab. Despite the reduction in immunosuppression, patients had stable renal functions without loss of graft functions. The patient with atypical Burkitt lymphoma had an abnormal karyotype, did not respond to treatment completely, and died due to disease progression. The other patients are still alive and in remission 5 and 3 years after diagnosis, respectively. EBV-negative post-transplant lymphoproliferative diseases are usually late-onset and are reported to have poor prognosis. Thus, reduction in immunosuppression is usually not sufficient for treatment and more aggressive approaches like rituximab with combined chemotherapy are required.

Keywords: Renal transplantation, Post-transplant lymphoproliferative disease, Lymphoma, Immunosuppression, Rituximab, Abnormal karyotype

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Epstein-Barr Virüs-Negatif Post-Transplant Lenfoproliferatif Hastalık: Tek Merkezden Üç Farklı Olgu

Şule Mine Bakanay¹, Gülşah Kaygusuz², Pervin Topçuoğlu¹, Şule Şengül³, Timur Tunçalı⁴, Kenan Keven³, Işınsu Kuzu², Akın Uysal¹, Mutlu Arat^1
1Ankara University School of Medical, Department of Hematology, Ankara, Turkey
²Ankara University School Of Medical, Department Of Pathology, Ankara, Turkey
³Ankara University School Of Medical, Department Of Nephrology, Ankara, Turkey
⁴Ankara University School Of Medical, Department Of Medical Genetics, Ankara, Turkey

Böbrek naklinden 6-8 yıl sonra Epstein-Barr virus (EBV) negatif posttransplant lenfoproliferatif hastalık geliştiren 3 olgu rapor edilmektedir. Hastaların tanıları gastrik MALT lenfoma, gastrik diffüz büyük B hücreli lenfoma ve atipik-burkitt lenfoma idi. Hem EBV early RNA için yapılan in-situ hibridizasyon yöntemi hem de EBV DNA için yapılan polimeraz zincir reaksiyonu ile doku örneklerinde EBV saptanamamıştır. Hastalara immünsupresyonun azaltılması ile birlikte rituksimab içeren kombine kemoterapi protokolleri verilmiştir. İmmünsupresyonun azaltılmasına rağmen böbrek fonksiyonlarında önemli bir kayıp gözlenmemiştir. Anormal karyotipe sahip atipik Burkitt lenfomalı hasta tedaviye rağmen hastalık ilerlemesi ile erken dönemde kaybedilmiştir. Diğer hastalar hala remisyonda takip edilmektedir. EBV negatif posttransplant lenfoproliferatif hastalıkların genellikle geç başlangıçlı ve kötü prognozlu olduğu bilinmektedir. Bu hastalarda immünsupresyonun azaltılması tek başına yeterli bir tedavi olmadığı gibi rituksimab içeren kombine kemoterapilerle daha agresif tedaviler gerekli olmaktadır.

Anahtar Kelimeler: Böbrek nakli, Posttransplant lenfoproliferatif hastalık, Lenfoma, İmmünsupresyon, Rituksimab, Anormal karyotip

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