Extramedullary disease can develop de novo, that is at diagnosis before any therapy, and the rate for that is somewhere around the 3 to 5 percent range from previous clinical trial studies and our own data that has looked at this question. What is more challenging is the higher incidence that we are seeing in heavily pretreated relapsed/refractory patients. Some of the recent registrational studies are up to 25 to 30 percent patients having secondary extramedullary disease...
Extramedullary disease can develop de novo, that is at diagnosis before any therapy, and the rate for that is somewhere around the 3 to 5 percent range from previous clinical trial studies and our own data that has looked at this question. What is more challenging is the higher incidence that we are seeing in heavily pretreated relapsed/refractory patients. Some of the recent registrational studies are up to 25 to 30 percent patients having secondary extramedullary disease.
It’s also important to note that extramedullary disease is distinct from paraskeletal disease, which is basically a tumor tissue that’s originating from a break in the cortex of the bone, and these tend to be biologically different. So defining the incidence of true extramedullary disease is crucial, but we anticipate that around 25 to 30 percent of heavily pre-treated patients end up having secondary extramedullary disease.
What we have previously noted is that from a matched cohort study, patients who have a 1q gain or amplification at diagnosis or a t(4;14) translocation tend to have a higher risk for secondary extramedullary disease. There have been no clearly defined risk factors for de novo extramedullary disease just because it’s a little tougher to study that population because of the smaller numbers, but I would guess that 1q amplifications and t(4;14) translocation seem to be the most prominent risk factors.
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