July 7: Getting You Up to Date

Okay, so the diagnosis is Blastic Plasmacytoid Dendritic Cell Neoplasm.

Yeah, I've been practicing on and off for 48 hours, and I still can't say it fast, even though I now know what all those words mean.

So, I'll give you the details, and in the next post I'll tell you about my experience with going home and googling Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN).

BPDCN is a very rare and aggressive lymphoma (I think that's what I'm reading) which usually will turn into a particularly aggressive form of Acute Myeloid Leukemia (AML). There's probably been less than 500 diagnosed cases, though I can't find any certain number.

The outlook for BPDCN was particularly grim until the last five years or so, with pretty much everyone dying within the first two years and a median survival time of 13 months. (Whoo whee! Wow!) BUT ...

The problem was that they were treating it like other leukemias. If you obtain remission with chemotherapy with other leukemias, you wait and see if the remission will last. If it doesn't, then you do chemo again, obtain a second remission, then do a bone marrow or stem cell transplant.

Not with BPDCN. With BPDCN, the first remission never lasts—in fact, it never lasts longer than 3 months—and you don't get a second remission. The patient dies, and he/she dies pretty quickly.

So now they know that with BPDCN you obtain remission with chemo (an intensive chemo designed for AML), the chances of which they refer to as "excellent," and then you do the bone marrow transplant.

Then ...

Then they don't know. It's been too recent, and there's been too few cases, so they really don't know what happens after that.

I know of several cases that are currently in remission after bone marrow or stem cell transplant. One's at 18 months (currently) and one was at 26 months when the medical report was written (I think in January). Both patients were doing fine, still in remission.

One older patient (over 67) made it 57 months (almost 5 years), and it doesn't appear from the medical paper that the person died of the BPDCN, but it's hard to tell from the abstract that I am able to read on Pubmed.

So, technically speaking, that's my situation. I have a pretty good shot with chemo and bone marrow (or stem cell) transplant, assuming we can find a donor, and then I become part of the medical testing to see how long BPDCN patients last.

Okay, now some practical info...

Concerning being a bone marrow donor:

I'll post more info on how to offer to be a donor as I have it. The doctor says I should type my brothers and sister first. My children are not much better hope than the average citizen.

Giving bone marrow doesn't mean giving all the marrow in your body. They pull a little bit from your hip, just like they did the bone marrow biopsy on me, and then they inject it right into my bloodstream (after they've made sure to destroy all my bone marrow through chemo). The bone marrow, having stem cells in it, then proliferates on its own, replacing bone marrow, blood, and everything.

Really, it's a pretty amazing process.

If you want to find out more about offering to be a bone marrow donor before I do, try marrow.org. I will, too, but it will be a couple days before I post anything.

I've had a lot of offers from people to have their bone marrow checked for a match. It's really wonderful of everyone to be so kind. I am happy to report that giving bone marrow's not that bad. It's very weird, having a needle poking around inside your hip bone. I've never felt anything like it, but it wasn't bad, and I was only barely sore the next day, though another donor once said she felt like she'd been kicked by a mule afterward.

Concerning certainty of diagnosis:

The pathology lab in California settled on Blasti Plasmacytoid Dendritic Cell Neoplasm as an official diagnosis, but the doctors at Vanderbilt want to verify it. They are looking at my cells under a microscope from both the bone marrow biopsy and my blood. They have not given the BPDCN their blessing yet, though I think it's likely they will. It's also likely that even if they don't, I still have something rare. I just didn't match any of the more common stuff.

Also, I've seen pictures of bumps exactly like the bumps on my back on the internet from other BPDCN patients. Further, to have lesions (the bumps) as your first symptom, followed rapidly by leukemia symptoms, is the norm for BPDCN. On the other hand, they make the diagnosis on exact chromosome abnormalities on particular blood cells (in this case plasmacytoid dendritic cells, which help identify pathogens in the blood), so that's what they're verifying.

Okay, now I'm going to go write another post about the harrowing experience I had the day I was told I have Blastic Plasmacytoid Dendritic Cell Neoplasm.