Post-transplantation lymphoproliferative disorder after liver transplantation: report of 5 cases among more than 550 liver transplants in Iran.

BACKGROUND

Post-transplantation lymphoproliferative disorders (PTLD) are a spectrum of diseases defined as polyclonal or monoclonal proliferations of lymphocytes which occur after solid organ transplants. In this study, we report our first experiences with PTLD following liver transplantation in Iran.

METHODS

We retrospectively analyzed five cases of PTLD which followed liver transplantation among more than 550 liver transplants in our center. Of these, three were pediatric cases and two were adults. The underlying causes were tyrosinemia, autoimmune hepatitis, and progressive familial intrahepatic cholestasis (PFIC) in the three pediatric cases. HCV hepatitis was the primary cause for cirrhosis in one of the adults and the other adult was labeled as cryptogenic cirrhosis. All cases, except for one, developed PTLD during the first year following liver transplantation.

RESULTS

Patients were diagnosed as PTLD, B-cell, MALT and Hodgkin-like (according to the WHO classification of PTLD). The three pediatric patients died despite discontinuation of immunosuppressive drugs and chemotherapy. Fortunately both adult patients, until now, are still alive.

CONCLUSION

The incidence of PTLD in our center is lower than previous reports from other centers (0.9%), with a 60% mortality rate and worse prognosis in the pediatric age group.