My father is diagnost since july 2010 with plasmacytoid Dendritic Cell Blastic neoplasms. He had a bump on his arm. This has been irradiated and had disappeared after treatment. Now he has been a few weeks about 60 bumps on his hole body but especially on his head and face. Wednesday back to the hospital to hear they are going to start any new treatment. I like to hear stories from other peers

Hi My 16 year old son was diagnosed April 2010. We underwent chemo, radiation, a cord blood transplant that did not work and then a stem cell transplant that did work in October. We just got the go ahead to go home in January. We have had a few bumps since but on the whole doing great. Would be happy to talk more in depth on our story.

I live in St. Petersburg, FL, diagnosed 5/2010. and had a difficult time finding an oncologist who knew anything about BPDCN. I was urged to undergo a stem cell bone marrow transplant by the staff at Moffitt Cancer Center in Tampa. I have decided against this procedure as there are too many risks involved and no guarantee. Underwent a series of chemotherapy (CHOP) in late 2010. I had remission for a month and a half but the lesions began to recur by the end of Jan. and have continued to increase (21 the latest count). My latest PET Scan was performed on 3/21 and I will find out if any infiltration has occured on the 30th. My advice to anyone fighting BPDCN is to have a positive attitude -- I'm living with it, not dieing from it. Good Luck to all and God Bless!

I was diagnosed yesterday! (July 6, 2011) I'm going to push my doctor to do the chemo, then bone marrow transplant option unless he's got good reason not to. I agree with Kenny K's attitude, though from what I've read he may well not be alive at this point. I'd love to hear from you if you are, Kenny! Any updates on others? I hear those under 18 don't face nearly the dire prognosis that older folks do, though I'm only 50 ... well, will be next week. Happy birthday, huh? Actually, I am taking it as a birthday present. A new ministry for the second half of my life!

I am sorry to hear about your diagnosis, you have a long road ahead, but it seems you definitely have the right frame of mind. My 16 year old is doing great, even being young it was pretty tough, reason being the first transplant failed. Second one was a breeze after the first. Yes kids seem to be better able to handle the extreme treatment, but to your credit you look like a guy that is in pretty good shape so hopefully you can handle the treatment a little bit easier. I can add a little bit more later, there is another great site for this disease. I am just on my way to work and directly after have to take my son back for a clinic appointment, so I will be away for a few days, but next week I will give you this other site name and chat more. Feel free to ask anything, I will try to tell you anything you have questions about. Kenny I hope you are doing all right!

Thank you, alleycat! I'll keep my ears on (as they say).

17 months post treatment and I'm still here and still recovering! 8 rounds of Hyper CVAD and a TBI followed by anautologous transplant. Below is the accumulated wisdom on our rare disease "A fellow sufferer's blog":http://yippee-leukemia.blogspot.com/2011/07/july-7-getting-you-up-to-date.html and his accumulated info webpage: http://www.christian-history.org/blastic-plasmacytoid-dendritic-cell-neoplasm.html another webpage from Lymphoma.net: http://www.lymphomainfo.net/questions/general-lymphoma-questions/blastic-plasmacytoid-dendritic-cell-neoplasm-formally-nk-cell#comment-2915 a link to PubMed for medical papers: http://www.ncbi.nlm.nih.gov/pubmed/ a search there for "blastic plasmacytoid dendritic cell neoplasm": http://www.ncbi.nlm.nih.gov/pubmed?term=blastic%20plasmacytoid%20dendritic%20cell%20neoplasm and a search there for "plasmacytoid dendritic cell neoplasm": http://www.ncbi.nlm.nih.gov/pubmed?term=plasmacytoid%20dendritic%20cell%20neoplasm Irish Lymphoma Guidelines on Diagnosis and Treatment of Malignant Lymphomas pdf: http://www.haematologyireland.org/clinical/default.htm Online book: TUMORS OF HEMATOPOIETIC AND LYMPHOID TISSUES: http://www.ncbi.nlm.nih.gov/books/NBK9562/ Online Book: Cancer rehabilitation principles and practice By Michael D. Stubblefield: http://books.google.com/books?id=_JaPlNYgXkEC&printsec=frontcover&dq=Cancer+rehabilitation+principles+and+practice+By+Michael+D.+Stubblefield&hl=en&ei=cMmNTvy2FM7B8QOfrKQD&sa=X&oi=book_result&ct=result&resnum=1&ved=0CDIQ6AEwAA#v=onepage&q=Cancer%20rehabilitation%20principles%20and%20practice%20By%20Michael%20D.%20Stubblefield&f=false

A few recent developments that may be worth looking at: "ASH Paper":https://ash.confex.com/ash/2011/webprogram/Paper37851.html "abstract":http://abstracts.hematologylibrary.org/cgi/content/abstract/118/21/2588?maxt "Stemline":http://www.stemline.com/company.asp "another new novel treatment":http://www.lymphoma.ie/Latest/Modified-Ecstasy-May-Effectively-Treat-Leukemia-Lymphoma-And-Myeloma "article about it":http://www.medicalnewstoday.com/articles/233070.php

From keeping up to date on Pub Med and searching blastic plasmacytoid dendritic cell neoplasm there, there is a rising trend for succesful remissions after high dose chemo (like hypercvad and chop umong others) followed by Total Body Irraidation and a transplant, (autologous in my case or donor bone marrow or cord blood). "Pub Med":http://www.ncbi.nlm.nih.gov/pubmed/ I have just had my 2nd anniversary post transplant.

Recent papers worth a look: "pubmed 1":http://www.ncbi.nlm.nih.gov/pubmed/22450587 "pubmed b":http://www.ncbi.nlm.nih.gov/pubmed/22455538 and this older one I just came across recently: "clinical advances":http://www.clinicaladvances.com/index.php/our_publications/hem_onc-article/2065/

Thanks for all the info, oldtree! I think people find this thread somewhat often.

More good news on a new drug for graft vs host disease just approved in Canada: "NYTimes article on Prochymal":http://www.nytimes.com/2012/05/18/health/a-stem-cell-based-drug-gets-approval-in-canada.html "Company info from Osiris":http://www.osiris.com/therapeutics.php

Just an update for everyone. I posted my first about a month ago. My 68 year old father is now ready for his stem cell transplant at the Huntsman Cancer Hospital in Utah. The doctors decided that he would not survive the fully myeloablative chemo that would kill all of his bone marrow prior to transplant, so they are doing a non-myeloablative preparation for his transplant with total body irradiation. He will be getting his stem cell transplant this Tuesday. There could be more complications after the stem cell transplant with the reduced intensity conditioning prior to treatment, but no one is really certain as it depends on the patient. We pray all goes well. As we have journeyed through this process since last April, many people have asked our family what they can do to help out. We tell them - Give Blood, we are going to need it, sign up to be a marrow donor to help out another, and finally, fast and pray for us, for strength, courage, and for good outcomes. Best wishes to the rest of you! May blessings pour down upon you!

Some very good news for us on this webpage: from "lls":http://community.lls.org/message/120623#120623 Re: Husband Newly DX W/ blastic plasmacytoid dendritic cell neoplasm (anybody heard of it?) CBCACJ 19-Oct-2012 12:47 (in response to oldtree) Sorry I am just responding to this! I wanted to report the very good news that yes, the SL401 targeted therapy at Scott&White in Temple, TX (directed by Dr. Arthur Frankel) put my husband back into complete remission! He did the treatment in mid August and today he is getting a 2nd round of Donor Lymphocyte Infusion from his sister to help boost his immune system as it reconstitutes. The targeted therapy was a 5 day in-patient treatment at Scott&White (MDAnderson is no longer offering the treatment) and the main side effects were fever and chills. He WBC was less than 200 when he began and withing 48 hours, it had jumped to over 1000 and his spleen was significantly less swollen. He was the first BPDCN patient to receive the drug and the second patient started treatment today. Dr. Frankel is more than happy to talk to other patients and I am happy to answer any questions. Just respond to this or email me at rebecca.wagner2@verizon.net. You also visit www.caringbridge.org/visit/camwagner The doctor mentioned is here: "Dr Frankel":http://www.sw.org/Dr-Arthur-E-Frankel the second patient to get remission with SL 401 p32chevy 13-Nov-2012 16:44 My husband is the second patient treated for BPDCN by Dr. frankel at Scott and White Hospital in Temple, Texas. His response to the drug was also successful. His recent BMBx came back clean. He also had skin lesions and they disappeared. My husband is 72 and still tolerated the treatment well. Please feel free to contact us also if we can answer any questions. . We can be reached at eorednick@gmail.com. It was on this blog that we heard about Dr. Frankel from CBCACJ and we are so very grateful. Stemline publish about their success with SL401 against bpdcn: "Stemline":http://www.stemline.com/newsArticleDetails.asp?id=47 I emailed Dr Frankel who is very approachable and part of his reply was: "I have treated two patients with BPDCN and both achieved a durable compete remission even after transplant relapse."

More positive news from the ASH conference this year: from a company in the UK called adaptimmune getting very positive results using extracted t cells then engineering them followed by a one off injection: "New Scientist":http://www.newscientist.com/article/dn22613-soupedup-immune-cells-force-leukaemia-into-remission.html "Adaptimmune":http://www.adaptimmune.com/news-events/

That SL-401 sounds awesome.

Yes it does, Its a great relief that there is another possible option, than the chemo and TBI we have already been through, for a relapse. The first patient ,Cam, is having a GVH problem with his liver, his bilirubin is up, but was off for a 60 day biopsy on the 20th. We wait to hear with fingers crossed for a good result for him. "cam's webpage":http://www.caringbridge.org/visit/camwagner/journal/1

More really good news in a very recent published paper on pubmed: Stem cell transplantation can provide durable disease control in Blastic plasmacytoid dendritic cell neoplasm (BPDCN). http://www.ncbi.nlm.nih.gov/pubmed/23203822 Three-year cumulative incidence of relapse, disease-free survival and overall survival was 32%, 33% and 41%, respectively. much much better statistics for survival than in previously published material.

Good to hear the news about recent data on survival rates. My father is day 137 out from his stem cell transplant. He is experiencing horrible graft versus host that is attacking his skin. He just had a relapse and experienced an abnormal amount of edema and hypothermia putting him into multiorgan and septic shock that has affected his kidneys. Christmas Eve in the ICU and since then. They are trying to help body temp and increase thyroid meds to help warm his core as he has been unable to maintain his temp or maintain conciousness. Still trying to find out the cause but no infections present. If anyone else experiences abnormally low body temp, edema, some fainting, muscle weakness, and has labs with high potassium, have the docs check the thyroid and other hormone levels. This would have saved us much grief over the past 6 weeks and less kidney damage by seeing if increasing the thyroid hormones would improve the symptoms and resolve the edema and potassium issues without multiorgan shock. SL401 sounds promising and so does the latest data on survival rates for this nasty one. Glad to hear oldtree and Paul are doing well. Praying for CAM and my dad and the Dutches. Haven't heard from Kenny for a while. Hope you are doing well also! Thank you for sharing your experiences through your blogs and this website. The information has proven very helpful as we have made this journey. Luck to all!

Dr-Arthur-E-Frankel has asked me to post the following on any BPDCN patients blogs who may be interested: "Dr Frankel":http://www.sw.org/Dr-Arthur-E-Frankel Arthur Frankel, cell number 254-718-0781, emails are afrankel@sw.org and arthur.frankel@utsouthwestern.edu The protein drug, DTIL3 or SL-401 is a fusion molecule containing the catalytic and translocation domains of diphtheria toxin and human interleukin-3. The drug was administered to close to 50 AML and MDS patients in a phase 1 trial at doses of 1-12.5ug/kg IV over 15 minutes daily for between five and fourteen days. There was a 10% remission rate and toxicities were vascular leak syndrome, acute infusion reaction, and transient transaminasemia. In a pilot study, two patients with BPDCN who were refractory to allogeneic stem cell transplants received DTIL3 at 11.5ug/kg for three to five doses over ten days and both patients achieved complete remission. Both remissions are ongoing at 2+ and 3+ months. Side effects were limited to the same toxicity profile previously seen in the AML/MDS phase 1 study. We are actively seeking several more BPDCN patients to confirm this activity. "Clinical Trial":http://www.drugs.com/clinical_trials/stemline-therapeutics-inc-announces-presentations-sl-401-updated-clinical-trial-results-acute-14810.html?utm_source=feedburner&utm_medium=feed&utm_campaign=Feed%3A+Drugscom-ClinicalTrials+%28Drugs.com+-+Clinical+Trials%29

the above mentioned paper is now available to read free online: "Stem cell transplantation can provide durable disease control in blastic plasmacytoid dendritic cell neoplasm: a retrospective study from the European Group for Blood and Marrow Transplantation" here: "Blood magazine":http://bloodjournal.hematologylibrary.org/content/121/3/440.full

Just thought I would you know that I have just reached my 3rd anniversary post transplant, woopee, but I still recovering most of the time but its is getting better slowly :-) (That would be about 3 years 10 months post diagnosis) Hang on in there! We'll beat this Blastic plasmacytoid dendritic cell neoplasm (BPDCN) yet!

Congrats Oldtree! I am so excited that you have reached this milestone! It is so wonderful to hear about this! I appreciate the updates and articles as well. We will beat this! Keep up the fight!

I just read the article from Blood Magazine that Oldtree posted three months ago. Once again, it confirms that a stem cell/marrow transplant in the first remission is the way to go and is remarkably successful (50% versus almost 0% for other treatments). What is discouraging is to read in their that reduced intensity treatment for BPDCN may not work. They had a small sample-size and mostly older recipients, but I think it's only older recipients for whom they try reduced intensity transplant.

The following is from my Histopathology Report in 2009 Clinical Data Disseminated lymph node enlargement and bone marrow involvement. Specimen Received Paraffin block Microscopic Description Lymph node showing a diffuse infiltrate of monomorphic cells with irregular, folded nuclei and small nucleoli. These cells are CD2 positive, CD3 negative CD5 negative, CD7 weakly positive, CD4 weakly positive,CD8 negative, TdT positive, CD56 positive, CD33 positive, CD123 positive, CD20 negative and CD 68 negative. MIB-1 shows a proliferication fraction of 50-60%. Histological Diagnosis Lymph node; blastic plasmacytoid dendritic cell neoplasm aka bpdcn I was lacking cutaneous involvement which is unusual for bpdcn. A proliferication fraction of 50-60% was a very poor starting point as I had to have an autologous transplant. I am now approaching 5 years post diagnosis and 4 years post treatment without a relapse.

Congrats, Michael! I'm over 2 years post-transplant now.

Congrats to you too Paul, onward we go banging our heads against the wall :-)

Hello to anyone reading this. I am still here in remission from blastic plasmacytoid dendritic cell neoplasm aka bpdcn Life is a bit of a struggle, but when isn't it? :-)

I'm at 3 years post-transplant from BPDCN and doing well, though I have minor GVH issues. I have moved on, diagnosed last month, to lymphoma. After I was diagnosed, I did a lot of research and found a Dec. 8, 2014 study showing survival stats for my lymphoma had improved from around 30% five years ago to about 80% because of a new chemo regimen. It turns out I didn't need to do all the research. That Dec. 8 article was the results of a phase 2 study conducted by doctors right here in Vanderbilt. The doctor in charge of my case told me about the study, and said their phase 3 study is showing a 97% survival rate treating Diffuse Large B-Cell Lymphoma with myc rearrangement with an regimen called EPOCH. She said part of the reason for more studies is they are having trouble believing such positive results. That's a lot better than the prognosis was for BPDCN. In fact, I was the one who changed the heading for this section of rareshare.org to match post-2009 studies. When I got here, the heading said that the prognosis for for BPDCN was successful remission with an AML-type chemotherapy regimen followed by "quick and fatal relapse." That was true in the early 2000's, but it is now standard to immediately follow a chemotherapy regimen with a bone marrow transplant and save a significant percentage of patients. Official 5-year survival rates are not statistically sigificant yet because the immediate stem cell transplant was not common until 2009, but I understand it's at least over 25% despite how many BPDCN patients are over 60. Word is that a lot of over-age-70 patients are surviving as well, some because of Dr. Frankel's SL-401 treatment which Oldtree mentioned in an earlier post.

Hi Guys, Glad to hear all is going well. I live in the U.K and my husband was diagnosed with BPDCN in July 2014. He had a stem cell transplant on 14th Nov 2014 and his brother was the donor. So far so good and he is home now although he has a slight g v h which is just a slight rash on his body. Prior to this he was very fit and cycled 100 miles per week on his bike, yesterday he managed 3 miles on his static trainer which was great, I firmly believe his fitness levels have helped him through this, along with his positive attitude, Keep up the fight. Take Care Elaine x

Hiya Elaine, Great to hear the news of another survivor. I believe that my fitness levels helped me survive both the disease and the treatment. I was a tree surgeon so spent a lot of time climbing trees :-) and was physically very fit. I was never areobically fit (not really a runner) and have ongoing breathlessness issues so even a few minutes on the static bike here and I'm finished for the day. "never give up" is such an important mindset! Michael x

Agreed. Those kind of fitness levels are awesome, and attitude has to be just as important. I had a nurse actually tell me, "You can tell who's going to make it by the way they act."

When I was diagnosed, I was told I had a "non-Hodgkin T cell lymphoma" called Blastic plasmacytoid dendritic cell neoplasm (BPDCN). I came across this article on the BBC that cites the WHO, saying "A specialist panel found sufficient evidence to link the chemical, already banned in the EU and the US, to a cancer called non-Hodgkin's lymphoma. *Lindane* is still used in some developing countries." "BBC article":http://www.bbc.com/news/health-33236293 Lindane is a persistant pesticide, like DDT it just keeps building up in the system. "WHO press release":http://www.iarc.fr/en/media-centre/pr/2015/pdfs/pr236_E.pdf "WHO website with this as top story":http://www.who.int/en/ I remember being asked if I had come in contact with any chemicals as the disease I had was NOT genetic. That it was not genetic was a great relief to me, meaning I had not passed it on to my children. Keep up the good fight and never drop the head :-)

Hi Michael, A very interesting article. I often wonder if my husband's diagnosis had something to do with anything he was given or exposed to in the British Army (he did 22 years) I hope you are well?. Nigel my husband had his transplant in November 2014, and is now back to working 4 days per week , although he does work at desk. He is back to riding his bike, I have to watch he doesn't push himself too hard but he can do around 15 miles a ride if he doesn't try and race his friends. He still has an appointment at the hospital every couple of weeks but everything is going in the right direction. Take Care Elaine x

Hiya Elaine, great to hear things are going so well. no way I could cycle 15 miles - the lungs just wouldn't take it. If fact I couldn't cycle 100 meters lol. I push myself to the limit with what I can do as I know no other way to do things :-) basically upper body stuff with little or no bending but I love being wreaked by doing it. It is a very interesting article and I too as a tree surgeon/gardener have to now wonder what I was exposed to over the years in ordinary gardens. These things build up in the environment and persist. Make sure he eats his fruit after you wash it :-) great to hear from you Michael x

Just came across contact info about the SL-401 Clinical Trail: SL-401 in Patients With Blastic Plasmacytoid Dendritic Cell Neoplasm "Clinical Trials. gov":https://www.clinicaltrials.gov/ct2/show/NCT02113982?term=SL-401&rank=1

Hi Michael, I hope you are still doing well, and trust this message finds you in good health. Am afraid about 8 weeks ago Nigel had a lump appear on his arm, in exactly the same place as the original lumps. I had a bad feeling as soon as I noticed it. We called the hospital and they had him go in straight away to have an emergency PET CT Scan, he had the results and they said they were cancer clear but he had arthritis (not nice but a huge relief as it was only arthritis) Anyway 4 weeks later he went back for his regular check up and saw a different consultant (the top bloke) who wasn't happy with the lump and sent him for a biopsy the same day. Sadly the dreaded BPDCN is back and he now has another on his leg. They have removed both. HIs scan showed something on his bowel also which they hope is a polyp, but they removed it last week and we get the results back on Wednesday. If it is BPDCN then he has to have more chemo. If that is just a polyp then its radiotherapy on his arm and leg and another stem cell top up from his brother. To say I am devastated is an understatement but Nigel is such a positive person so I remain positive (in front of him anyways) and I guess we will just take each day as it comes again. Take Care Elaine x

Elaine, I am so sorry to hear this! I will pray for him. I have no advice for a situation like that, but I will pray someone will come along who can give you good advice. Have you checked into the possibility that Dr. Frankel's SL-401 is worth trying?

Hi Elaine, Yes still here fighting away. I'm really sorry to hear the bad news. Yes you will have to be very strong for Nigel, he will need you to be his rock in a hard place. I will have my fingers crossed for Wednesday. Love Michael x

Hello Michael. I have been reading peoples stories on this blog and you seem very knowledgeable and active on this disease. My husband and I are Canadian. Bob, who was 73 at the time was diagnosed with BPDCN in late February 2021. He had hyper-CVAD and a stem cell transplant in June 2021. He was doing remarkably well until he got two viruses, RSV and Epstein-Barr, at the same time. This was September 2021. The RSV almost killed him but the rivpbavirin treatments given in a negative chamber environment worked and he survived. He was also treated with rituximab for the Epstein-Barr.
Bob’s blood counts plummeted after this and he went for months with no immunity, blood transfusions and platelets. In March 2022, he received IVIG and his counts jumped up immediately. Unfortunately, the day before IVIG he was having confusion and trouble walking and when I mentioned it in the clinic at his IVIG infusion, they did a brain scan and found a tumour (I believe in the right temporal lobe). After multiple tests it was diagnosed as BPDCN. He is now receiving intrathecal chemo for the tumour and azacitidine injections to keep his body in remission. The docs say his tumour is in remission now. Bob’s health seems to be declining rapidly with bouts of confusion, memory loss and severe weakness. I will be phoning his doctors tomorrow, July 10, 2022 to report this decline which has been about a week.

Still here and ageing quickly 

Had my lenses in my eyes replaced due to cateracts from the radiation treatment a few years back, and have recovered from a pulmonary embolism and collapsed lung last October. Hyperbaric treatments really helping out the lungs and curbing the chronic fatigue.