Post Transplant Lymphoproliferative Disorder

Patient Presentation:
E.R. is a two-year-old female with a history of biliary atresia and failed Kasai procedure with a subsequent liver transplant. E.R. also had a past medical history that included acute rejection post transplant that was treated and resolved with a week of a high dose steroid taper and increased levels of the main immunosuppressant, tacrolimus. E.R. presented to the emergency department with fever of 38.1 axillary. E.R.’s mom asserted that E.R. has had 2 weeks of fevers that come and go with no accompanying symptoms. All other vital signs were WNL. E.R. had blood and urine cultures drawn. Upon assessment, E.R. had noted lymphadenopathy. E.R. was admitted to the pediatric transplant unit for further care.

Diagnosis:
E.R.’s blood and urine cultures were all negative. Meanwhile, E.R.’s fever resolved within 24 hours. The week prior to this admission, E.R. was seen in the transplant clinic and regular transplant labs were drawn. Results found a high Epstein Barr-virus level. When EBV infects an immunocompromised transplant patient, it can lead to uncontrolled proliferation of EBV infected B cells resulting in B cell lymphoma. This diagnosis is referred to as post transplant lymphoproliferative disorder. For E.R, the high EBV levels, lymphandenopathy, and fevers of unknown origin gave cause to obtain a PET-CT. The results were lymph node enlargement and an extranodal lymphoid tumor on the liver. A biopsy was obtained to confirm PTLD.

Treatment:
After the confirmed diagnosis of PTLD, E.R.’s immunosuppressant drugs were decreased so she could better fight off the PTLD. E.R. was also given infusions of rituximab, a monoclonal antibody that destroys B cells. E.R. had these infusions administered intravenously once a week for four weeks. In E.R.’s case, the rituximab was sufficient to resolve the PTLD and put her into remission. In other cases, more intense chemotherapy drugs can be required if rituximab does not work.

Outcome:
E.R. had to be closely monitored as an outpatient because the decrease of immunosuppressants during the course of her PTLD treatment had put her at a higher risk for rejection of the organ. Therefore, her current immunosuppressant drugs must be fine tuned to prevent rejection but also ensure that she is not over immunosuppressed.