No abstract

The amyloidoses are a rare group of disorders caused by the deposition of amyloid fibrils in organ tissues. Transthyretin-mediated amyloidosis is a subcategory of amyloidosis, which can be further subdivided into hereditary transthyretin-mediated or wild-type (‘senile’) amyloidosis. Involvement of the prostate by any type of amyloidosis is rare, with only a handful of cases reported in the literature. We report here a case of a 64-year-old Caucasian man who was incidentally found to have wild-type transthyretin-mediated amyloidosis of the prostate following a radical prostatectomy for treatment of a localised, Gleason 7 (4 + 3) adenocarcinoma of the prostate. TTR subtyping was confirmed by mass spectrometry. His family history was negative for known amyloidosis, and sequencing of the entire TTR gene was negative for any mutations, so he was therefore considered to have wild-type TTR amyloidosis. He was subsequently found to have additional foci of amyloid deposition in the gastrointestinal tract. Wild-type transthyretin-mediated amyloidosis as an incidental finding in a prostate biopsy is an extremely rare finding. Treatment options for transthyretin-mediated amyloidosis are limited, and further research and treatment options are needed in this rare and difficult to manage disease. Level of evidence: The level of evidence according to the Oxford Centre of Evidence-based Medicine levels of evidence is not applicable to this case report. Given that this is a rare entity, decisions made in managing this case were based on a level of evidence of 5.

No abstract

There have been significant improvements in therapeutic options for relapsed multiple myeloma (MM) over the past two decades, with many novel agents including proteasome inhibitors, immunomodulatory agents, and more recently monoclonal antibodies demonstrating efficacy in this setting. However, there is a paucity of real-world data comparing outcomes seen in patients treated with novel agents as opposed to older agents. We report a historical single center cohort of patients diagnosed with myeloma between the years 1991–2012 in order to explore possible differences in outcomes. A total of 139 patients who underwent stem cell transplantation were included in our study. In our study, 88 patients were treated with cyclophosphamide and steroids alone at relapse whereas 51 patients were treated with Len-Dex. In the multivariate analysis, TTNT was shorter for patients who received Cyclo compared to Len-Dex (HR = 1.74; 95% CI, 1.01–2.99; p = 0.04); however, we could not detect an overall survival benefit (HR = 1.20; 95% CI 0.63–2.29; p = 0.57). Adverse event rates were similar in the two groups. In this retrospective single center analysis, Len-Dex was associated with longer TTNT compared with Cyclo at first relapse following autoSCT in MM; however its effect on overall survival in this setting was less clear.