How long does someone live with BPDCN?
So this is one sad and difficult part of BPDCN and why we're gathering today, and why I've dedicated my own life and career to it. I was quite shocked when I first got into this field approximately 15 to 17 years ago. At that time, BPDCN's name was just starting to become codified and really abysmal. Overall survival, I would say around eight months from the time of diagnosis to the time of death, either transformation to acute leukemia, progressive BPDCN itself, or unfortunately infection, multi-organ failure, sepsis, kidney failure, etc. Usually older patients, a lot of comorbidities, including maybe even another cancer.
And a lot of these folks over time, that overall survival, we believe and hope that we were starting to double, maybe triple, hopefully in some of the series as we add the treatments we'll talk about here, targeted therapies, potentially some cytotoxic multi-agent regimens giving CNS prophylaxis, going for an early stem cell transplant, when available, etc. But I would say it's a sobering reality that the historical overall survival, this is in frontline BPDCN, it used to be eight months, maybe up to 12 months. Maybe now we're a little bit more than that. And then the relapse refractory, you know, less than six months would be the overall expectation.
Again, these numbers are historical. They're sobering. They're very unfortunate. But that's what the original medical is. That's why we're doing this interview. That's why we're writing these papers. Investigating is that we need to urgently look for new approaches and new hope for our patients and families facing this rare disease. Each case is somewhat individualized. You know, if you look at the literature, they'll say somewhere between 8 and 12 months is the median survival.
And of course, you know that that leaves a lot to be desired, both to do better, but also, you know that that doesn't really inform the individual what their outcome is going to necessarily be. So the outcome is also obviously dictated by the ability to receive therapy. That's often influenced by whether someone is elderly and frail or has other comorbidities. And then, as we mentioned, transplant—if transplant is an option—that's potentially a curative option that could significantly improve survival.
So it's a moving target. We know it's a chronic progressive and aggressive disease. And it can limit life. But with the therapies we have at hand, we like to think that we can improve that and even cure with transplant.
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