blastic plasmacytoid dendritic cell neoplasm (Formally NK Cell Leukemia/Lymphoma) Ever heard of it?

Hi All

My husband was diagnosed with this in April & I am just trying to find out if anybody has heard of it? It is very rare they tell us.

For Vicki,

Please tell me what has transpired between July 09 and now (Oct. 10). My older brother was just diagnosed with this.

Hello,

My name is Kyle and my 79 yr old grandfather was just diagnosed. This guy moves around like you would not believe. To me, incredibly healthy. His Doctor thought he may have 1-3 mos, maybe 6, at most a year. He was told only 200 people in the world have this. He also told me his doctor knew nothing of this cancer and actually had to look it up on the internet?!
We are from Temperance,MI just north of Toledo OH. Are these the same findings as yours? How is your husband? Is he stable, regressing dramatically like told? Hard for me to wrap my hands around. Please reply.

.

My grandfather was diagnosed with CD4+/CD56+ in Jan 2010. His inital symptom was a red papular lesion on his chest. He went to his family physician who gave him triple antibiotic cream. None of us thought to much about his lesion until he showed us about 6-8 months later. The lesion had now tripled in size and had irregular borders. We were refered to dermatology and the biospy reported CD4+/CD56+ (natural killer cell lymphoma). We were referred to a local Oncologist. After many tests he decided to have local radiation to his abdominal area. Apparently one of the lymphomas was causing him minimal discomfort. After he finished his radiation, it was found that it had no affect on his lymphoma. My grandfather is 83 years old and has no pain or discomfort with this cancer. The only thing that really bothered him was the appearance of the lesions that had now spread to his face and to his entire torso. It seems like the tumors that were on skin really started to spread about a month after the radiation treatments, however he still was not having any other symtoms.

As of Spetember 2010 my grandfather started to become very fatigue. His oncologist said that because of his age, he didnt really recommend chemo. He told us that the side affects were too risky and probably not worth the illness that would follow. He did leave this decision up to my grandfather. Grandpa opted to have the chemo, but backed out last minute. The night before chemo he had severe abdominal pain. We went in to see his oncologist the next day, and they said that his spleen was enlarged thus the pain. The pain subsided after one day? Since then we have called Hospice and I am currently living with my grandfather. My grandmother (his wife) died Apeil 2008 of stage 5 pancreatic cancer. We lost her after 3 weeks of finding out about her cancer. This was a very cruel and painful cancer...my grandfathers is nothing like hers AT ALL. His main sysmtoms now are extreme fatigue, severe shortness of breath, bloated appearance of the abdomen and his skin is very sensative. As of yesterday I think I noticed a change in his skin color. He states that he is not in pain and doesnt feel like he is dying? I still am doing research on this rare form on cancer, but from what I am reading it appears that the leukemia is getting the best of him.
None of the doctors know very much about this cancer...it has a 1:100,000 ratio. They say that it is not heriditary??? After watching my grandmother go through so much discomfort I guess that I am greatful that he is not in pain...

My stepfather was recently diagnosed with this cancer. He developed a small lump on his chest, and after it enlarged, he visited his primary care physician to have it removed. A slice of the tissue was sent to a major hospital for testing. The result of the biopsy indicated that the features were that of this cancer we're discussing. He has been to a hospital that specializes in cancer, and had a blood test, bone marrow test, and PEP scan of his entire body. This past week (11/22/10) he met with the specialist to find out the results. The doctor said the cancer is in his skin (which is good, since it hasn't spread), but that they saw "some activity" (I suppose a gentler way of saying, "it's in") in the bone marrow. So next week, he will be in the hospital for 10 days of chemotheraphy, along with bunch of drugs, which the doctor said will be instense, and could be fatale...so they need to keep constant watch over him during this period. The doctor said they will need to find a donor for a bone marrow transplant. Unfortunate for him he does not have siblings, so that complicated matters, as far as finding a match. They would like to do the bone marrow transplant within 30 days, and said that if they cannot find a match, that they will continue with the chemo. What is strange is that this man is feeling perfectly fine, with no pain anywhere, and his appetite is good. I have done some research on bone marrow transplant, and the impression I'm left with is that the success rate is almost non-existent. All I'm left with is that this man will die of this cancer in the very near future. Please share your experience with me if you have been (or going) through this process. I'm trying to learn as much as possible about this cancer, its treatment, impact, and the eventual outcome. Thank you in advance for sharing your experience.

After a strong fight with this awful cancer, my grandfather left us to join his wife with our Lord and savior November 21st @ 7:44pm. Nothing that Hospice gave us was true about this cancer. I hope and pray that there can be a cure for this form of cancer for all of of you out there.
Good luck to you and your family Brava...the only regret that I have about this whole thing was allowing my grandfather to do the radiation first. This seemed to be the catalyst that allowed this disease to consume him. I am sure that the outcome would have been the same...but maybe we could of had my grandfather longer.

Krissi,

I am so sorry to hear about your Grandfather. To be blind-sided by such a fast and furious attack leaves us numb, doesn't it? WIth all the info about breast cancer, colon cancer, lung cancer -- name it -- we have no reference point on this "awful cancer," as you say. Lymphoma? Leukemia? What the hell is it????

My brother is being treated in Southern California, and I'm seeing a more aggressive attack on this cancer there. I'm also seeing an ability from his docs to change daily as to the protocol. He is having a "big guns" chemo intravenously, a periodic chemo injected into his spinal cord to prevent a spread to the cord and his brain -- and he is preparing for a bone marrow transplant in January. There seem to be 60 possible donors at this point from the bone marrow registry. (Even though I, as his sister, should be a good possibility, I am not a match.)

I'm not sure radiation has even been planned at this point. And he seems to be doing better than expected with the current regimen. My brother is also choosing from various anecdotal remedies to fight the symptoms and side affects from chemo, and having good luck with those. I will elaborate if there is interest out there.

Nancy

Krissi,

Thank you for your prayers.

My brother is 62. He was diagnosed around the first of October this year. He does not complain nor elaborate on the spinal cord treatment. I think he is grateful for anything that might help.

He has mentioned something that I find interesting: As the chemo begins its work, he said he is realizing how bad he felt before. He thinks he might have been harboring the disease for a year or so before the skin lesions sent him to the dermatologist for a biopsy.

We hope and pray for a miracle. He is surrounded by good friends and family, and we are grateful for that.

Nancy

I was diagnosed last july 2009 with non hodgkin's t cell lymphoma plasmacytoid dendritic cell neoplasm, but with some issue over immature t cells. My symptoms were a swelling of the lymph system rather than a rash. It was amazing when i started the treatment and the nodes felt like thay were bursting and the pain ebbing away.
I was 44 and very healthy, and it was felt I could endure the kitchen sink being thrown at me. There was no reason why it happened, dont drink or smoke, veggi.
My treatment consisted of 8 rounds of high dose hyper c vad chymo. I did not match a sibling donor nor the European donor database, so I got an extra small dose of chymo to stimulate my own stemm cells which were then harvested. I then had 2 days of three times the precious doses of chymo followed by a TBI, at a rate of 7 (1 or 2 is usual for tumors etc), followed by the transplant of my own stem cells.
Following a pet scan and bone marrow biopsy I was given the all clear in August 2010. The full all clear is unknown as this is such a rare entity, so who knows, neverminding the consequences of the TBI.
My recovery is very slow, and feels slower as I wes a very active and physical in my work. I have been told it may take another year or so to become normal. My bloods continue to improve.
My vacination programme is due to commence in May 2011, till them I must be very careful.
I feel very lucky to be here and have just celebrated my 2 childerens birthdays in october and also christmas with them. I managed to take them and the dog for a walk today.
Stay positive, dont panic, engage brain and endure. I am a very different person now after 191 days in hospital, but the unswerving support of my family cannot be underestimated, nor my will to spend more time with my children.
I have just started an appetite stimulant (side effect of Megestrol Acetate) as I was finding it very difficult to eat, lack of saliva and appitite, and within 4 hours was making up for lost time.
My brithday is the 21st November.....

I was so happy to finally see that someone had even heard of this cancer. My 13 yrs old son was diagnosed in Oct. 2008. It started with a tumor on his leg (side of the calf). It was like a blue bruise. He had been being kicked by a bulling girl in the legs everyday at school but one of the bruise would not go away so i took him to the doctor. They immediately sent us to a surgeon who took out the tumor. It was sent to the States because they could not figure out what it was here in Canada. 2 months later they told me it was cancer and they think they got it all. Two months later another tumor was in his groin. They then decided on chemo treatments after a CT PET scan showing he had tumors everywhere. We were all tested for a bone marrow match in the family which was not found. He was put in the bone marrow bank till they found a 10/10 match in Feb.2009. After 5 months of high dose chemo we went for the transplant. After a 4 month stay in the hospital we went home and he has been great ever since.

My brother was diagnosed with this in October, 2010. He has, in the ensuing months, received 2 (successful?) chemo treatments and a bone marrow transplant April 12, 2011. He is home and, of course, in quarantine. But although he is weak, his spirits are up and we are hopeful. So far, all he is experiencing is the "normal" host vs. donor" rashes and being very careful.

Do not give up if the bruises/bumps reappear. The docs are gaining information every day, it seems like. I think my brother might be one of the longest survivors of this particular type of lymphoma.

He is being treated by the City of Hope in Duarte, CA. He is convinced he has lived this long because of them.

Nancy-
I'm glad to hear that your brother is doing well. Was his transplant autologous or allogenic?

And regarding City of Hope, only recently- within the past six months or so- they performed their 10,000th stem cell transplant. There may be no better medical center in North America for this procedure than City of Hope.

Ross

My 16 year old son was diagnosed with Blastic Plasmacytoid Dendritic T Cell Neoplasm in April 2010. At the time, like so many of you, no one in Winnipeg Mb had ever seen, treated or diagnosed it. Our Oncologist sent all my sons results to Vancouver, Toronto, Seattle, Mayo and France. When all feedback came back they decided to carry out France's treatment plan that consisted of maximum chemo and radiation followed by a BMT. At the time there was no donor matches but they found a 4 out of 5 cord that transplant was done in July and failed. In Sept a 10 out of 10 stem cell donor cropped up on the list and agreed. His stem cell transplant was on Oct 8/2010. So far it has been very successful. GVHD has been fairly controlled and other then the Shingles virus wreaking havoc on his Nerves, we are doing fairly well. After staying in Winnipeg 10 months our family was able to come home to Ontario in January this year. He is still fairly weak but getting stronger each day, they expect physically it will be a year to get back to normal and they expect 2 years before his immune system is good, they are talking about starting his vaccinations next month. I was so happy to find this site and interact with others who have experienced this brutal disease. When we were diagnosed the prognosis was so poor, but through the last year , it seems they are making some headway and I am finding some success stories.

It is good to hear that other people are in recovery. As far as I'm concerned every day is a bonus.

I also got the flu vaccine before winter and was well until christmas when 3 colds knocked me off my feet. I was put on a monthly infusion of Kovic (an immune booster) that worked wonders and I felt great too. As usual though I reacted to it and was taken off it after 3 months. No more colds thank goodness.

However high blood pressure raised its head after christmas and I have just had a kidney biopsy to see if it is the result of scarring or some other reason. The tablets for it make me feel very lethargic on top of the continuing cronic fatigue.

One other side effect of the treatment has persisted which is numb feet that are hypersensitive. Looks like thats here to stay but I can't complain really.

I am now off all the other meds and no vacination programme was deemed necessary as the transplant was my own stem cells, so onwards and upwards.

I hope to go for a swim this year when on holiday.

I did also feel like I created a a bit of a monster and we all do those thing for our children because we knnow if they did not make it we had to live with it all. I remind my son sometimes that the cancer is gone and we are back in the real world with our second chance at life. It is hard for us because we went through the whole cancer journey on our own his dad just left me and our family was toren apart and his dad did not ever step up to help. Now there is the anger and frustration of divorce i am trying to protect him from. But it is still nothing compared to the painful recovery of cancer. I am just grateful still I got to bring my child home.

That all sounds very hard to deal with, but stay focused on your children nomatterwhat. And to be blunt (which I have found myself to be moreso than I was) what dosn't kill you will make you stronger. The whole experience has sorted out the wheat from the chaff with regards to people worth knowing.

I am still exhausted all the time but try to push myself but can pay a very heavy price for even going two steps beyond what my body is capable of, and expect to be like this for another year at least.

Last week I was very tired on tuesday so spent a lot of time in bed. Felt great on wednesday so went out into the garden without a jumper on and did a bit of hedge cutting, wrecked but felt great. so then i went out to sweep up some glass on the road from a crash. Was like a zombie on thursday got a sore throat followed by rigor and high temps for two days and am now on antibiotics for an infected sinus hoping that it wont go down into my chest. Its a very tough balancing act and after a year you would think I would have learnt! :-)

Feet are also swelling up and I found pressure stockings (Teds)are good but nothing beats my pair of lace up walking boots for keeping the swelling down. I protect my feet with thick fleece socks that are both warm and no lines of irritating stitching (cant wear wool socks for instance).

Silcocks Base cream is excellent for retaining skin after TBI or rashes or dried out splitting skin.

I cannot imaging what impact the treatments would have on children and would not wish the experience on my worst enemy. Just surviving is a huge trauma. At times I feel that half my brain/memory/self has been erased or changed by the treatment and I'm not coming back.

Patience and endurance and love and understanding. slowley slowley catchee monkey or monmster in this case.
As long as the monsters come back to us when they are adults as reasonable human beings thats all that matters for any parent.

ps it will be 5 years at least before I could be called cancer free and that will always be on my mind
mx

17 months post treatment and still here!

A fellow sufferer's blog:

http://yippee-leukemia.blogspot.com/2011/07/july-7-getting-you-up-to-dat...

and his accumulated info webpage:

http://www.christian-history.org/blastic-plasmacytoid-dendritic-cell-neo...

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%20dendrit...

and a search there for "plasmacytoid dendritic cell neoplasm"

http://www.ncbi.nlm.nih.gov/pubmed?term=plasmacytoid%20dendritic%20cell%...

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=Can...

Hello All,

I just posted on the LLS boards as well so forgive the repeat but I have never been on this page before and wanted to join the discussion. Unfortunately, today we got the news that my husband's BPDCN relapsed after 32 months(his BMT was March '09). We are devastated and shocked because he still feels great and has been healthy for 2 1/2 years, living a full life. We know we caught it early b/c he had a clean biopsy in September and we thank God for His timing in all that. For now, we are overwhelmed with the decisions for next steps and know we are in unchartered territory since they just started doing transplants for BPDCN as the primary course of treatment around my husband's diagnosis. In other words, I don't think there is anyone alive who has survived relapsed BPDCN and that is terrifying. The current plan is more chemo with donor lymphocyte infusion from his sister (the donor). His tranplant was a "mini" or reduced conditioning since she was only a half match, or haplo-identical so they made need to go for a fully ablative transplant now.

For now, I will praying for all of you warriors and caregivers out there and we welcome your prayers too! God's grace is sufficient and will carry us through this.

Never loose hope - God is good...ALL the time,

Becky

ps - we are actively seeking 2nd medical opinions so if anyone has any suggestions or recommendations, please let me know.