Current recommendations of diagnosis and treatment of posttransplant lymphoproliferative disorders

Abstract

Posttransplant lymphoproliferative disorders (PTLD) are uncommon but a serious complication
after solid organ or hematopoietic stem cell transplantation. The incidence is the highest
during the first year after transplantation and varies according to the type of transplanted
organ, type of immunosupression and status for Epstein-Barr virus (EBV) infection. The
majority of PTLD belongs to high-grade B-cell lymphomas, EBV-positive. The transformation
of B lymphocytes usually by EBV infection uncontrolled by T cells is responsible for the development of PTLD. However, more EBV-negative cases have been noted recently. It is
estimated that PTLD occurs after a heart transplant in 1.0–6.3% of patients (pts), lung in
4.2–10% pts, heart and lungs in 2.4–5.8% pts, kidney 1–2.3% pts, liver 1–2.8% pts and as
many as in 20% in patients after small bowel transplant. Treatment of PTLD begins with
reduction of immunosuppression. If no response is achieved within 2–4 weeks, pharmacological
treatment is necessary. Here we present and discuss a protocol of PTLD treatment recommended
by the Polish Lymphoma Research Group, consisting of a sequential administration of
rituximab in monotherapy and in combination with CHOP chemotherapy.