A 22-year-old female with a history of bilateral lung transplantation for cystic fibrosis 9 months ago now presents with fever, weight loss, and lymphadenopathy. Lymph node biopsy shows diffuse large Bcell lymphoma.
Treatment includes all of the following EXCEPT:A. Reduction of immunosuppression
Correct Answer: B
Posttransplantation lymphoproliferative disorders (PTLDs) are lymphomas that occur after solid-organ or allogenic HSCTs due to immunosuppression and infection or reactivation of Epstein-Barr virus (EBV), though up to 50% of cases of PTLD may be EBV-negative.
Reduction of immunosuppression is the first-line treatment of PTLD. Calcineurin inhibition should be reduced by at least 50% and antimetabolic agents stopped. Since response to immunosuppression reduction occurs early, restaging of lymphoma can be performed at 2 to 4 weeks. For patients who do not have a response to reduced immunosuppression, rituximab may be added. Chemotherapy, radiotherapy, surgical excision, and adoptive immunotherapy may also be indicated depending on the response to reduced immunosuppression and rituximab, and the subtype of PTLD.
Since PTLD is associated with EBV-seropositive donor organs transplanted into EBV-seronegative recipients, prophylactic ganciclovir and EBV monitoring may reduce to risk of PTLD.