Blastic Plasmacytoid Dendritic Cell Neoplasm

Blastic Plasmacytoid Dendritic Cell Neoplasm:
Why There's Hope!


In July, 2011 I was diagnosed with Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN). I am writing this page to spare you the shock that I experienced when I searched this horrifying version of leukemia (almost lymphoma) on Google.

Let's begin by saying that web pages on BPDCN present a hopeless outlook. Ten years ago, that was an accurate outlook. Today, BPDCN is cured almost as easily (and regularly) as Acute Myeloid Leukemia (AML), to which it is related.

Admittedly, that's not a very good prognosis. AML survival rates are still only around 30%, and if you're diagnosed over the age of 70, it's worse because treatments, as well as your ability to survive the treatments, are more limited.

NOTICE!! I AM NOT A DOCTOR!


I am a fellow patient. My leukemia turned out to be an Acute Undifferentiated Leukemia (AUL) related to Blastic Plasmacytoid Dendritic Cell Neoplasm, but I did a lot of research on BPDCN. Even now, the treatment for my AUL is affected by its similarity to BPDCN.

I have read medical papers published on the PubMed database concerning BPDCN. I have run my conclusions by my doctors at the Vanderbilt Medical Center in Nashville, Tennessee in the process of determining my treatment. The information I'm giving on this page is well-researched, and I have been very careful to make sure it's double-checked and accurate. Nonetheless, I am not a doctor.

This is meant to be helpful, hopeful, and very accurate (as of 2011) advice for fellow patients. You will not survive Blastic Plasmacytoid Dendritic Cell Neoplasm without being treated by a competent doctor in an excellent medical facility.

Contact the Leukemia and Lypmhoma Society to ask about financial options if you do not have insurance and cannot afford treatment at a good hospital.


Here's Why the Web Sites Are Wrong and You Can Survive.


Most web sites that pass on the awful prognosis normally associated with Blastic Plasmacytoid Dendritic Cell Neoplasm are basing their information on old data.

Paul Pavao with juice and bald from chemotherapy for acute undifferentiated leukemia

Juicing in between chemotherapy treatments, hoping that maybe really good nutrition would help me handle the whole process. You can't juice during chemo because raw vegetables have bacteria that are dangerous when blood counts are low.
Years ago, perhaps even as recently as 2006, BPDCN was treated like other acute leukemias. Chemotherapy was given to put in remission, and then the doctors would wait to see if it would stay in remission.

Unfortunately, unlike other acute leukemias, Blastic Plasmacytoid Dendritic Cell Neoplasm almost never stays in remission. It always relapses, usually in under one year, and then the patient dies. It appears to me from reading the medical papers that no one has ever successfully treated BPDCN after a relapse.

This is different than almost all other acute leukemias. With other acute leukemias, you can achieve a second remission. Not so with BPDCN.

Therefore, once BPDCN is put in remission by chemotherapy, a stem cell transplant must be performed within a few months!

In the last few years, as medical professionals have discovered that a stem cell transplant (or bone marrow transplant) must be performed, hospitals have begun complying, and BPDCN victims are beginning to survive on a regular basis.

The problem is that survival rates for leukemia are based on a 5-year period. Most of the people properly treated for BPDCN have been cured in the last three years. Thus, the 5-year survival rates are still appear awful.

I am in contact with a man in North Carolina who has been clear for three years. I am in contact with another man in Ireland who has been clear for 16 months (as of October, 2011).

There are many others. One of the studies that looks awful is one by Dalle et al. The abstract says, "Only eight (17%) and one (2%) patients reached respectively 2 and 5 years of survival."

Sounds terrible, right? However, if you purchase the entire paper published in the British Journal of Dermatology, you find out that only ten of the cases they studied included a bone marrow transplant. The paper is old enough to include cases in which the doctors did not know how mandatory a transplant is for BPDCN sufferers.

Of the ten people transplanted, six of them were still alive at the end of the study. On average, they had been alive for two-and-a-half years (31.3 months). One of them had already survived 77 months.

Blastic Plasmacytoid Dendritic Cell Neoplasm is still a terrible disease. The older you are, the more terrible a disease it is because the treatment could kill you even if the disease does not. But it is not the certain death that a Google search would seem to indicate.

More Helpful Information


Please email me if you know of other sites that might be helpful.

  • You can talk to other Blastic Plasmacytoid Dendritic Cell Neoplasm patients and survivors at the Leukemia and Lymphoma Society's community. That link will take you directly to a thread on BPDCN, but you can search for other messages there as well.
  • There's a set of messages at lymphomainfo.net. The link takes you directly to the message by Michael, the fellow in Ireland who has survived BPDCN for a year and a half since transplant. The whole page is about BPDCN, though.

My Shocking Google Search


I described my experience with googling "blastic plasmacytoid dendritic cell neoplasm" on my Thrilled to Death blog. Here I'll just give you the list of quotes I found on the sites that came up on the first page of my Google search.

  • "Despite the initial response ... being mostly excellent, the prognosis is poor"
  • "this aggressive hematological disorder"
  • "which is VERY RARE [caps in original] and according to the Internet not curable and usually presents with skin lesions [note: I have these]. There are very few cases reported and not very good outcome."
  • "Systemic dissemination and short survival are characteristic." [Keep in mind here that "systemic dissemination" means having leukemic symptoms, which I have.]
  • A very rare aggressive cancer that manifests in skin lesions ... No long term treatment has been developed ... to date. Life expectancy is estimated at twelve to fourteen months. Chemotherapy offers a short term remission (approx. three months). A bone marrow transplant extends lifetime up to three years.
  • The prognosis ... is generally poor with a median survival of 14 months. The treatment is conventional chemotherapy for AML, which generally shows a good initial response followed by QUICK AND FATAL RELAPSE.

I wrote this page because quotes like that took my breath away when I read them on the internet. I seriously hope that this page will make it onto the first page of Google so that others will get a more up to date account of Blastic Plasmacytoid Dendritic Cell Neoplasm.

What the Heck Does Blastic Plasmacytoid Dendritic Cell Neoplasm Mean, Anyway?


The words in Blastic Plasmacytoid Dendritic Cell Neoplasm actually mean something:

Blastic
A blast is an immature cell. The defect in Blastic Plasmacytoid Dendritic Cell Neoplasm occurrs at the immature stage of the development of the blood cells.
Plasmacytoid Dendritic Cell
Plasmacytoid dendritic cell (pDC) is the name of a type of blood cell. There are also myeloid dendritic cells in our immune system. The pDC's, however, are similar to plasma cells, so they are called "plasmacytoid." I don't understand their function real well, but basically pDC's hang out mostly in the skin, nasal passages, and other areas exposed to the outside world. They recognize dangerous invaders ("pathogens") like viruses and present them to T-cells (and B-cells?) for destruction.
Neoplasm
A neoplasm is a tumor. Most often, the first indication of BPDCN are lesions on the skin. These are actually tumors.

Thus Blastic Plasmacytoid Dendritic Cell Neoplasm basically means "A tumor caused by immature dendritic cells of the plasmacytoid type."

My Experience Somewhat Briefly


You can follow my entire experience here on this blog, but here's the short version.

Long about late March of 2011, I developed a small bump on my back that I thought was a bug bite. It itched a bit. I thought it went away, but a couple weeks later I noticed it was still there but bigger.

About a month later I developed another very close to the first. By late april, the first bump was almost the size and shape of a Florida palmetto bug—about an inch long, half inch wide, quarter inch high, and roughly oval.

In May, my wife told me the bumps were blue. I had not realized that. They were in the middle of my upper back, not a spot that's easily visible even in the mirror. (At least not for a 49-year-old. I suspect I was able to turn my head more when I was younger.)

I had been trying to get in shape ever since a doctor gave me a physical back in September (2010). My blood counts were normal then, but my blood pressure was barely high. The doctor told me to lose 30 pounds.

I lost 10 pounds in the fall, and I was running 2 miles at a time. I slacked off over the winter as I really didn't seem to be progressing, which I chalked up to being overweight. The discouragement and the cold weather led to me taking a couple months off. I was pretty sure once I lost some more weight, my running would improve. (That's acually what happened when I took up jogging at 40, too, so experience backed up my assumption.)

It wasn't until May that I began to realize that not only was I not getting better, I was getting worse.

I never dreamed that something drastic was wrong with me. I am prone to harmless cysts. I have had a number of them, and I was sure that was the issue with my back.

By June the bumps on my back had not only become larger, but they no longer looked like cysts. The skin had become bumpy and had turned a rust color, rather than blue and smooth as they had been.




We never got a picture of the lesions at their worst. This is in June sometime. In early July, the top one grew so quickly that it tore my skin, leaving a scab that can be seen in the picture below, after the chemotherapy shrunk the tumors to nothing.

Blastic Plasmacytoid Dendritic Cell Neoplasm lesions

Blastic Plasmacytoid Dendritic Cell Neoplasm lesions

So on June 21, 2011 I went to see my doctor. Even with the bumps in the condition they were in, he thought they were cysts. We even scheduled an appointment for the following week to have them removed by a surgeon.

On June 24, however, he called me back in to tell me that my blood test showed that I was anemic. He had looked at my blood under a microscope, and all my lymphocytes (a type of white blood cell) were large and abnormal. My white blood cell count was almost double what it should be, yet my neutrophils (the type of white blood cell that fights bacteria) were barely visible under the microscope.

He told me he was certain I had leukemia.

I went to Corinth, MS for more tests the following week, and they took a bone marrow biopsy. The following week the doctor told me that I had an acute leukemia, but that my bone marrow was so unusual that he wanted to turn me over to a bigger hospital. He specifically recommended two doctors at Vanderbilt University.

The first week of July I went to Vanderbilt, where I was told that the pathology lab in California (yeah, my bone marrow went to California) diagnosed me with Blastic Plasmacytoid Dendritic Cell Neoplasm. Even though the doctor told me that Vanderbilt would have to confirm this, I began to research BPDCN, assuming that the pathology lab couldn't be wrong.

The following week, the doctor informed me that Vanderbilt's pathology lab refused to confirm BPDCN. My leukemia was like BPDCN, but it didn't quite have all the markers.

I have done enough research on Blastic Plasmacytoid Dendritic Cell Neoplasm to know why the diagnosis wasn't confirmed. I had "markers" for CD4 and CD56, which are the prime markers for a BPDCN diagnosis. I was also strongly positive for CD123 and TCL-1, which are also "suspicious for BPDCN."

However, I had a strong positive for CD10, which is more like Acute Lymphocytic Leukemia. Also my CD56 positivity was light, and it usually didn't concur in the same cells as the CD4 positivity. I believe the negative for MPO was also unusual.

Just in case there's anyone reading this who understands all that.

Anyway, they switched my diagnosis to Acute Undifferentiated Leukemia, and they put me in the hospital on July 14. Chemotherapy began the next day.

Acute Undifferentiated Leukemia means that my leukemia is acute, but they can't put it into any of the typical leukemia classes. AML and ALL, which are Acute Lymphocytic and Acute Myeloid Leukemia, are the most typical acute leukemias.

Acute leukemias are more deadly in the short term. AML only has an average survival rate of about 30% at 5 years, taking everyone into account. Keep in mind that the average age of diagnosis for AML is 67, so those above that age are a lot less likely to survive for 5 years than a 30- or 40-year-old.


Acute leukemias move very fast. By July 12, three days before chemo began, it was difficult for me to walk up even the four stairs leading into my house. I would pause and catch my breath to do so. My spleen was so enlarged that I had to sleep with body leaning to the right to make more room on my left side.

The lesions after they shrunk. A scab is left on the top one where it tore in early July from rapid growth.
Blastic Plasmacytoid Dendritic Cell Neoplasm lesions

The chemo, however, was powerfully effective, shrinking the tumors on my back to completely flat in four days. I had tumors developing on my right collarbone and inside my upper lip. Both of those disappeared in a couple days, though the one on my clavicle (collarbone) left a small brown mark. The ones on my back have left large—but flat and smooth—brown marks on my back.

A bone marrow biopsy on July 28 and another around a month later showed me to be fully in remission. I had a "consolidation" round of chemotherapy early in September. This is typical to help make sure that all the leukemia cells are destroyed and to ensure that the remission continues.

The rest of the story is complicated, and as I write this (Nov. 2011), I'm still about a month away from being able to get my bone marrow transplant. They've found leukemia cells in my spinal fluid. I've had a mild heart attack, despite a good heart, no artery blockages, and no damage done by the heart attack itself!

This is my journey, turned over to God, who has never failed to produce excitement and something unusual at every turn. At the price of pain, I have made friends, helped people, received help, and learned more than I could ever have imagined.


For "the rest of the story," just follow the blog!





26 comments:

  1. Hi,
    I am grateful to read your story. My brother is just dinogsis the "nk/t lymphoma" and the doctor said is just like what I can find on Internet, very rare, poor response to chemo, 7% survive rate in 5 years......... Looks hopeless. Reading your blog gives me some hope. Although doctor said he is too weak for a bone mellow transplant for now, if he can survive the first chemo, there maybe a chance for him still. he just started chemo few hours ago, will last for 3 days. God bless him.
    And wish you a health living.

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  2. Thank you, Milk. I will pray for your brother, and I'm sure those who read this will, too.

    As a bit of info, Blastic Plasmacytoid Dendritic Cell Neoplasm used to be called several other things. NK/T lymphoma has to do with the "Natural Killer" cells and T-cells, and it's called a lymphoma because of the skin tumors. It is, however, the same thing as BPDCN.

    The reason for the problem is that in BPDCN, the malignant (cancer) cells go bad so early in development that it is hard to tell what they were supposed to become. Calling it NK/T Lymphoma supposes that those cancer cells were trying to become NK-cells. Calling it BPDCN means the researchers think they were supposed to become Plasmacytoid Dendritic Cells.

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  3. Thanks! Will pray you stay in remission. I'm a skin pathologist in the UK and was googling the condition as the books don't say that much about it; we get occasional cases through our department. I think the tumor can show inconsistent markers on the biopsy, but CD4, CD56 and CD123 are characteristic. Glad they made the right diagnosis in the end, and sounds like you're getting good treatment. Will remember what you said about the prognosis being based on older published papers.
    Best wishes,
    Nick.

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  4. Please look into a public company _ Stemline Therapeutics - STML that is in FDA stages for BPDCN and AML

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  5. Hi I am to read this story we are from Ireland and my dad has been diagnosed with this in march 2013 ! After reading some horror stories on goggle I'm glad that there is some hope for this type of cancer ! He has been told he is the 2nd person in Ireland to have this type of cancer thankfully through out all his treatment his bone marrow has stayed clean thank god and is currently waiting for his stem cell transplant ! Best wished to u in ur recovery

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  6. Email me at paul@christian-history.org & I will put you in touch with the other Ireland survivor.

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  7. I just watched your video. It's astounding what the mind will embrace to appease it's fears. You speak "faith" but there you sit in a hospital bed in the hands of science. You say god wants you to live, yet you go through all these procedures...so were is this faith? and why bother if it's all in gods hands?

    I've just watched two young girls die from leukemia in the last six months, they also prayed to their god who ignored their prayers and that of thousands of other that prayed for them. So to what end were they taken so young? Did god need them to praise him in heaven? Does he just desire the parent suffer in anguish for their lost the rest of their lives?

    I'm truly sorry you have the illness and hope you recover fully, but your willingness to reject reality and embrace a fantasy simply shows you will believe anything to appease those fears, as have countless others praying to whatever god they believed in over centuries past. All those other gods you reject.

    Best of luck, and the best to good science.





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  8. If those are real questions, and not rhetorical ones, then I will tell you my answer. Let me know.

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  9. thank you for writing this page. I came across is on page one of a google search for bpcdn, and I am grateful that I did.

    My mom just told me she has been diagnosed with it, and reading google is horrifying. thank you for giving me some hope

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  10. Thank you, Kat. I am glad it helped. Your comment is the very reason that I wrote this page, to provide real hope and counter the old information that is on the web.

    I'm going back for my 2-year bone marrow biopsy. No reason to suspect relapse, and my doctor tells me they treated me so harshly that he's as sure as he can be medically, which is never 100% certain, that I won't relapse at this point.

    I have a friend at 3.5 years, and I have a new 65-year-old acquaintance being treated in Kentucky for BPDCN. He's doing great entering the second round of chemo, and the expect to be able to give him a stem cell transplant even at his age.

    Also, look up the Lexe Selman story. She had AML, not BPDCN, but she's playing college soccer less than two years after her diagnosis, even sooner than that after her bone marrow transplant.

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  11. Hii, I am from India. My friend Abhijit 26 years of age has BPDCN. He went for bone marrow transplant but it failed. Then doctors gave him autologus transplant after which he showed signs of improvement. He was completely healthy and had no cancer cells for two months. But again it has relapsed and doctors are saying that he may or may not respond to chemotherapy again. I am feeling helpless...

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  12. Hi Darin. I answered you on Google +. If I need to contact you some other way, let me know. My email address is paul@christian-history.org. If you can't get help from my friend Michael in Ireland, I have other suggestions.

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  13. Hi Paul, Thank you so much for your story! My father was just diagnosed yesterday. Although the stains all suggest he has BPDCN, the doctors are not yet certain and are waiting for second pathology reports. The up side to all this is that after the ct scan mri it appears there is no spread or growth and they believe they found it in early stages.

    Funny thing with all of this is that it seems to be affecting myself and younger brother more than my parents. We are both well educated and studied biology at what I would consider great schools in CA. The problem so far is that I can't find any papers or discussions that dont have a bias or account for bone marrow transplants. Luckily, my brother is a donor match and has taken a courageous step and volunteered to help but the hard part is that he is finishing up his last semester of his undergrad courses.

    I guess, the point of this response is how did/do you talk to your loved ones? How do you stay positive through this whole process? I know you mentioned some support groups earlier in the article, but how do you let yourself open up to these "strangers" (and by no means am I implying they are bad, but merely not familiar)?

    I have taken steps to meet with oncological nutrition specialists to help get my dad on a better diet these few days we have leading up to chemo. Are there any other recommendations you have? What about encouraging people to talk to? My dad tries to be that "idealistic male" by which I mean, nothing scares him (although i know he has to be feeling something), everything is fine just rub some dirt on it sort of guy. Any ideas on how to break through and get him to open up? The mere fact you put together this site for people to find positivity and hope makes me feel like i was somewhat destined to find this site!

    I truely believe he can fight and win this but I know he is going to need the help and I want to be able to have a team of support there ready and waiting for him. Here's my email address: jermblake@gmail.com, I would love it, if you have the time, to talk and discuss this with you.

    I really appreciate what you are doing here and wish the best for you and your family! Please keep up the great work. Hopefully I will hear from you, I'm very much so looking forward to it!

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  14. Hey Paul, my name is Shauna i have subscribed to your blog. Since my mom has been diagnosed with BPDCN she is undergoing chemo and will be staying in the hospital for the next 28 days she is positive and I try my best to also stay positive and assure her that she will beat this. I have been trying everything I can to help her and I just can only hope that she will be one of the survivors!

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  15. My friends husband has just been dx with BPDCN and just started treatment, he is 66 and asymptomatic apart from the lesions on his back. He is only the second person in Scotland I believe. I saw Bob Cleland the other man's posts but not any very recent so wondered if he is still well and if it is possible to contact him? I was relieved to see that the prognosis is not quite as bad as Google suggests. Thank you.

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  16. I've gotten in touch with Shauna and Carol42 by email & FB.

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  17. Paul my 15 year old son was diagnosed yesterday. We are at Children's in DC. As it is rare, there are not many examples here. We are debating to stay or find a more experimental course. I believe here is standards chemo for 3 years. The first 30 days in hospital. The rest out patient. With possible stem cell from sibling. As this just happened yesterday, any advice?

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  18. As far as I know, standard chemo for three years will kill your son long before the three years is up. The usual treatment for BPis an induction round design for AML, followed by finding a donor for A marrow transplant. The transplant should wait no longer than 6 to 9 months. A 15-year-old will have a good chance of being able to handle. In the end, the only side effect he would end up regretting here's is never being able to go out in the sun without sunblock again..

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  19. Sorry for the typos. I was on my iPhone. You should get a second opinion from a hospital that has done a lot of marriage transplants. Please feel free to email me. My email address is in the comments above

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  20. Hi Paul, Thank you so much for sharing your story. My dad was diagnosed last week and i was feeling hopeless until finding this page. He is at the mayo clinic in Minnesota today going through treatment options. Thanks again for bringing hope to so many people and sharing all of the information that you provide on this page!

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  21. Thanks Matt. I'm glaid I can be of any help. I am three years out now and still alive. A lot of us don't make it, but 5-year-remission and a declared cure is no longer unusual. Every year doctors learn more. We're on the upswing in this battle. There's a treatment available now called SL-401 that's an option when chemo is impossible or when it fails. Keep your hope up. Also feel free to email me anytime at paul @ Christian - history . org. You have to take the spaces out to use that address, but don't take out the dash or period.

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  22. Hi Paul, I hope you are doing well. My mom is actually doing great. She will have her 2 year spinal tap next Monday. How are you?

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  23. Hi Paul,

    My boyfriend, 31, was recently diagnosed with BPDCN in august. He did 4 rounds of SL-401, but we were just told yesterday the cancer is still progressing. They are now going to start chemo and radiation, in order to hopefully get him in remission for the BMT.

    I just wanted to say thank you for sharing your story. It gives me hope!

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  24. Is this site still active? It's 2017, Day 0. Will I make three years out? Someone please respond, anyone.

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    1. The site is not active, but I still am! Day 0 has to mean you are getting a marrow transplant today, right? Has it happened yet?

      I got a marrow transplant on January 12, 2012, and I am still here. Chin up. You can do this, and yes, you can make it to three years and even to many.

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