Bortezomib-based induction for transplant ineligible AL amyloidosis and feasibility of later transplantation.

Recent studies support the use of bortezomib-based therapies in light chain amyloidosis (AL). We performed a retrospective analysis of the safety, efficacy and long-term survival (median follow-up 3 years) after bortezomib-based treatment in 28 consecutive patients with de novo AL deemed ineligible at initial presentation. The first 14 patients received bortezomib and dexamethasone (VD), and the second 14 patients received cyclophosphamide, bortezomib and dexamethasone (CVD; CyBorD). Both regimens were well tolerated with no treatment-related mortality. The overall hematological response (HR) rate was 93% in both the groups. Median time to response was shorter in the CVD group (39 days vs 96 days in the VD group; P=0.002). Hematological and organ responses induced with bortezomib-based therapy enabled 8 (33%) of initially transplant ineligible patients to undergo autologous hematopoietic stem cell transplantation (AHCT), including 4 patients with cardiac stage III or IV. Seven of the eight patients (88%) who underwent subsequent AHCT achieved sustained HR at a median of 33 months posttransplant. These data suggest that bortezomib-based induction followed by AHCT is a viable therapeutic strategy for transplant-ineligible AL. Larger, multicenter prospective trials are necessary to confirm our findings.

Large granular lymphocytic leukemia presenting late after solid organ transplantation: a case series of four patients and review of the literature.

Post-transplantation lymphoproliferative disorder (PTLD) is a significant complication of solid organ transplantation. Most PTLD is of the B-cell subtype, although T-cell subtype PTLD uncommonly occurs. T-cell PTLDs are usually aggressive neoplasms and shorten patient and allograft survivals significantly. We present a single-center case series of 4 patients who developed T-cell large granular lymphocytic (LGL) leukemia, a rare T-cell PTLD characterized by large granular lymphocytes that have characteristic azurophilic granules and a highly variable clinical course.