Hi, my name is Danae Dimon. I'm an assistant professor of medicine at Fred Haas Cancer Center in Seattle. And today I'm going to talk about our work that is focusing on outcomes and management of patients with MGRS, which means monoclonal gammopathic renal significance. So basically MGRS is a series of renal disorders that are caused by the deposition of monoclonal immunoglobulins or fragments of those immunoglobulins in the kidneys. And these monoclonal immunoglobulins are produced by plasma cell population in the bone marrow and less commonly by maybe a lymphoplasmicidic population or a B cell population. But that population is not large enough to meet the criteria for a hematologic malignancy or did not meet the criteria for treatment actually. So basically these patients have no other indication to be treated or to basically being told that they have cancer. It's just the renal impairment that basically drives the therapy and all the management decisions. There's a lot of heterogeneity in terms of histologic subtypes. They're different ones based on what disease they're linked to. And sometimes they can be related to amyloidosis, but because amyloidosis is a different entity in our research project, we have decided to exclude those patients and also exclude patients with active multiple myeloma who have renal involvement. So we only included patients that have what we call non-amyloidosis and GRS. So this was a single institution retrospective analysis and then included 31 patients with MGRS. The most common histologic subtype was PGN-MID, about 50%. And 40% of our patients had what we called nephrotic range proteinuria, which means they had a lot of protein in their urine. And the median EGFR that describes kidney function, a diagnosis was 30, which is actually kind of on the lower side and that's expected because this patient's had renal impairment. So most of the patients received traditional therapies for their MGRS and that included mostly bortezomib and cytokine. We saw 40% of our patients received the immunotherapy drug deratumumab and then other agents such as lenalidomide and rituximab were used for a smaller number of patients. So the overall renal response rate was 58% with first line therapy and another 20% of our patients a stable disease with first line of therapy, which was pretty good actually, pretty encouraging data. So 10 patients, which was about one third of our cohort progressed and they required second line therapy. And of those patients, half of those patients required third line therapy. A total of seven patients in our cohort received bone marrow transplant and we had one patient that received the renal transplant without any further progression of their disease after the renal allograft was placed. Then we went ahead and we looked at progression free survival outcomes when it comes to real progression free survival and we found that at two years, 77% of our cohort did not have renal progression, which was pretty good. And the median renal progression free survival was not reached. However, when we did further analysis, we found that having the histologic type of PGN-MID and nephrotic range proteinuria were adversely associated with the progression free survival. So overall, if you take home points for this disease, it's important to remember that MGRS is a very heterogeneous disease. And right now there's no standard of care, first line or second line management, and there are no official guidelines on how to treat this disease. So basically all decisions are institution dependent or physician dependent. Therefore we think that it's really important that further research should focus on developing biomarkers that will help us in selecting the right patients for the right therapies and also that will guide us with regards to what the right duration of therapy is. And then another thing is more efforts should be focused on having larger data sets that will help us actually create guidelines to standardize the management of MGRS.
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