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.
My understanding of your husband's cancer is that yes, it used to be known as ANKL (Aggressive Natural Killer Cell Lymphoma) but is now considered something a bit different: Now it's considered an agranular hematodermic neoplasm (HN), and your searches for information will likely produce more information if you search by that term.
It was also called blastic natural killer cell lymphoma; the reason it was changed was because it was found to derive from a plasmacytoid dendritic cell, which is a subset of the cells in our immune system.
Now, the following information on NH comes from the source cited in italics directly below this paragraph. I don't normally cite such large sections of studies like this but fear I might mislead you if I don't quote heavily:
Niakosari, Firouzeh; Monalisa Sur,. "Agranular [CD4.sup.+]/[CD56.sup.+] hematodermic neoplasm: a distinct entity described in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas." Archives of Pathology & Laboratory Medicine. College of American Pathologists. 2007.
[CD4.sup.+]/[CD56.sup.+] HN has a distinct clinical presentation of primary skin lesions in 100% of the patients, as papules, nodules, or bruiselike lesions; bone marrow involvement with or without leukemic phase; and a fatal course. The skin lesions are localized at onset and become multiple and more generalized during the course of disease. Transformation into leukemic phase occurs, if the patient survives to reach this phase.
The median survival time has been 14 months, with no difference in patients presenting with or without concurrent extracutaneous involvement.
On routine hematoxylin-eosin staining, the skin biopsy shows features of a high grade undifferentiated hematolymphoid malignancy with a differential diagnosis that includes:
1. Acute myeloid leukemia (AML)
2. Human T-cell lymphotropic virus 1 associated adult T-cell leukemia/lymphoma
3. Cutaneous NK/T-cell lymphoma
The prognosis for HN is generally poor with a median survival of 14 months. There is no difference in survival of patients presenting with or without concurrent extracutaneous disease.
The treatment is conventional chemotherapy for AML, which generally shows a good initial response followed by quick and fatal relapse in extracutaneous sites such as bone marrow.
Failure of conventional chemotherapy implies the necessity of developing alternate therapies, such as immunomodulation or immunotherapy with IL-3 or with anti-CD123 antibody.
That study cited the following studies:
(1.) Adachi M, Maeda K, Takekawa M, et al. High expression of CD56 (N182CAM) in a patient with cutaneous CD4-positive lymphoma. Am J Hematol. 1994;47:278-282.
(2.) Chaperot L, Bendriss N, Manches O, et al. Identification of a leukemic counterpart of theplasmacytoid dendritic cells. Blood. 2001;97:3210-3217.
(3.) Patrella T, Comeau MR, Maynadie M, et al. Agranular CD4+ CD56+ hematodermic neoplasm (blastic NK-cell lymphoma) originates from a population of CD56+ precursor cells related toplasmacytoid monocytes. Am J Surg Pathol. 2002;26:825-862.
(4.) Patrella T, Meijer CJLM, Dalac S, et al. TCL1 and CLA expression in agranular CD4/CD56 hematodermic neoplasms (blastic NK-cell lymphomas) and leukemia cutis. Am J Clin Pathol. 2004;122:307-313.
(5.) Karube K, Ohshima K, Tsuchiya T, et al. Non-B, non-T neoplasm with lymphoblast morphology: further clarification and classification. Am J Surg Pathol. 2003;10:1366-1374.
(6.) Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphoma. Blood. 2005;105:3768-3785.
(7.) Kato N, Yasukawa K, Kimura K, et al. CD2-Cd4+CD56+ hematodermic/ hematolymphoid malignancy. J Am Acad Dermatol. 2001;44:231-238.
(8.) Petrella T, Bagot M, Willemze R, et al. Blastic NK lymphomas (agranular CD4+CD56+ hematodermic neoplasms). Am J Clin Pathol. 2005;123:662-675.
(9.) Vermi W, Facchetti F, Rosati S, et al. Nodal and extra nodal tumor forming accumulation ofplasmacytoid monocytes/interferon-producing cells associated with myeloid disorders. Am J Surg Pathol. 2004;28:585-595.
(10.) Massone C, Chott A, Metze D, et al. Subcutaneous, blastic natural killer (NK), NK/T cell, and other cytotoxic lymphomas of the skin. Am J Surg Pathol. 2004;28:719-735.
Unfortunately, I can't find much else that relates to NH. It's possible that the Journal of Experimental & Clinical Cancer Research may be a good resource?
Please tell me what has transpired between July 09 and now (Oct. 10). My older brother was just diagnosed with this.
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.
hi my name is bob and i am recovering from this rare cancer which most of us cant repeat never mind understand so its no wonder there are a lot of questions to be asked and fears to be eased.I have come through all the chemo,lumber punctures and biopsies (not unscathed)but glad to be alive,i had my own stem cells cleaned up and put back in, which i"m glad to say has been very succesful one day at a time, i know that may seem simple but it was more complicated than that,thank GOD for the doctors and nurses of ward b7 BEATSON CANCER HOSPITAL in GLASGOW,they were brilliant and down to the grace of GOD,the power of prayer i am alive today.I believe I am the first case of this cancer in Scotland,possibly UK,to my knoledge.I was told by my consultant we"re going for cure Mr CLELAND,that was music to our ears I can tell you,my wife and I were over the moon with that prognosis considering the aggresivness of the cancer.I try to be grateful every day(not always sucsessful)but I"m trying,it"s not easy,so if anyone is going through this hang in, keep praying and GOD BLESS. BOB CLELAND.
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.
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...numb is a very good word. I am flooded with so many emotions and still have so many questions about this disease? And yes it does seem that in this day and age we would have some better treatments and answers for this form of cancer.
Please keep us posted about your brothers treatment.To have chemo in his spinal cord seems so invasive and sounds very painful.
How old is your brother and when was he diagnosed(if you dont mind me asking)?
You and your brother are in my thoughts and prayers...keep up the good fight.
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.
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 previous 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.....
HI,my name is bob and I"ve just read your experience with this terrible illnes,I too am recovering and in remission thank GOD,I"m a little older than you at 56 and like you was as strong as an ox until MAY 2010 when the cancer struck.I was told by my own quacks (and I mean quacks)that I had a hair folical on the side of my face which seamed quite plausable until 6 months later and several hospital visits the lump on my face grew to 4" dia.when finaly they took bone marrow and bone biopsies which turned out to be this very aggresive and rare cancer,and was told it had to be treated very aggresively,what a shock eh!you know the feeling.I went through the treatment fairly well until the final chemo which emptied my immune system and a viral pnemonia set in, that nearly finished me, thanks be to GOD,the power of prayer and the fantastic care and attention of the doctors and nurses i pulled through, oh!and the love of my fantastic wife, five daughters and three grandsons whom I love very much.I have just finished my new immunisation program thank GOD,I told the nurse now I know why kids cry after getting these jags.It would be great to meet up some time and compare experiences,who knows.Anyway I hope your doin well and this reaches you,GOD BLESS BOB CLELAND.ps.my phone number is 01555772885 call anytime.
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.
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.
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.
Your story is the same as my son's I felt so out of touch with other moms sometimes when he was in treatment because no one had his type of cancer I had no one to compare him too as in things to watch for or even know if he was going to live. He has been cancerfree for 2 and half years. We are doing his live vaccinations now. He had lots of trouble with rashes also. Probably from the radation Total Body Irradation has to be hard on the skin his feet and hands peeled off for weeks after. He is a normal kid again. He had a 10 out of 10 donor a 41 year old woman we have never met. He had a slight bit of GVHD but it went away quickly thank goodness. I am glad your son is doing good. Jesse's immune system was full strength in a year. He was on antibotic for a year 800 mg a day. He takes a little longer to get over colds and flus but he does get a flu shot every year.
Wow our stories are so alike, My sons original tumor was a bruise in his bicep, he was on the wrestling team, we never thought much of it. Within 3 days of diagnosis they operated and removed it. When results came back on the tumor they hauled us back to Winnipeg. He had his first immunization last week with the rest to follow in October, except the live ones. I am so glad your son is doing well, this gives me hope that we have beaten this! It is so great to hear success stories, I mean when we were told about the disease and I started researching it, the same prognosis was very grim everywhere I looked. My son is only on Septra now two days a week. He had drug toxicity and had to take him off all meds even the tacrolimus. They were scared of renal failure. But he is doing good without it and his GVHD is not acting up. So far so good. Thank you for replying it is good to be able to talk to someone that went through the exact same thing.
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.
You are absolutely right, every day is a bonus. I have also learned not to worry about things I can not control. Every day is a blessing, after being away from home for 10 months and leaving my other two kids with my parents, I have learned to not sweat the little things and there is no need to worry about the bigger things that might happen. You just deal as you go along. I am glad to hear you are doing so well, my son also has issues with his feet. One is numb, we had to buy Orthotics for his shoes to help give some extra support. I am glad to hear your feelings, sometimes I give my son a hard time of wanting to sleep all day now that we are at home, but listening to you makes me think Am I pushing to hard? Its hard finding a balance, He is 16 and just seems to lay in his room on the computer or gaming systems all day. I have been pushing him to get out and live, start doing normal things. Sometimes I just question how far to go, After the last year , he is quite used to being served hand and foot, and is quite spoiled. Our BMT clinician says we create monsters (giving them everything they want while this is all happening)but hearing about your chronic fatigue and how long it is taking you maybe I should be more patient. Thanks for your input!
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
17 months post treatment and still here!
A fellow sufferer's blog:
and his accumulated info webpage:
a link to PubMed for medical papers:
a search there for "blastic plasmacytoid dendritic cell neoplasm"
and a search there for "plasmacytoid dendritic cell neoplasm"
Irish Lymphoma Guidelines on Diagnosis and Treatment of Malignant Lymphomas pdf;
Online book: TUMORS OF HEMATOPOIETIC AND LYMPHOID TISSUES
Online Book: Cancer rehabilitation principles and practice By Michael D. Stubblefield
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,
ps - we are actively seeking 2nd medical opinions so if anyone has any suggestions or recommendations, please let me know.
I'm sorry to read of your husband's relapse. Regarding second opinions, considering how rare his tumor is, you would benefit most from a multidisciplinary second opinion, meaning an independent team of doctors reviews all his records and offers opinions on everything from biopsy results to treatment recommendations. Far and away the best list I know of is maintained by RA Bloch Cancer Foundation, the link to that incredible list is HERE.
Best of Luck-
I hope things have turned around for your husband and family, Becky.
A few recent developments that may be worth looking at:
Has anybody tried to contact Cancer Treatment Centers of America. They have one in Chicago, Philadelphia, Arizona etc..look it up..they advertise that they have medicines and treatments that not every doctor knows of. I would give them a try..google cancertreatmentcenters of america..
The main page for CTCA is www.cancercenter.com. Yes, they advertise that they have medicines and treatments that not every doctor knows of, but that doesn't mean they have more effective medicines or treatments. I'm not saying CTCA is good or bad, they are a privately run health network and while they have a very solid philosophy about how they approach treatment-- patient-centered, something the founder developed-- they are also a for-profit hospital network. When on commercials you here that their results outperform the national averages, the only reason this might be true is because when calculating national averages, CTCA is sure to include community hospitals and free county hospitals where the treatments and outcomes in cancer have been proven to be severely sub-standard when compared to hospitals associated with the National Comprehensive Cancer Network.
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).
I have just had my 2nd anniversary post transplant.
Congrats on two years post xplant oldtree, that is just awesome!
You're right about the flood of papers on pubmed about this subtype. I couldn't help but notice this one (http://www.ncbi.nlm.nih.gov/pubmed/22450587) in which an older patient experiences 21 months of remission (and counting) from a regimen traditionally used for Hodgkin's lymphoma-- ABVD.
This is the one that caught my eye recently:
and this older one I just came across recently:
Unfortunately all the papers we've listed involve case studies. There won't likely be any treatment guideline advances until someone can carry out a controlled prospective study of the disease and the treatments.
Thats very true Ross, these specifically selected good news papers are not guidelines but I feel the emerging picture is one of indicators and hope and that in of itself is a very important thing, compared to the dismal picture of a few years back when I was diagnosed (2009).
Given the rareity of this disease, somewhere around 250 or so worldwide, the chances of a controlled prospective study seems slim to me.
Wow the disease is that rare? Then I agree with you completely, a prospective trial is likely not forthcoming any time soon. Perhaps some researchers, maybe at Cochrane, can do a retrospective study of all the studies on the disease and come to some sort of conclusion. Just 250, that's unbelievable.
This recent case series and literature review was interesting to me as it covered my symptoms, ie non cutaneous (lymph nodes):
They find 27 non cutaneous and 188 cutaneous in their search.
They state 17% to 2 years and 2% to 5 years, gotten from this more limited review here:
They also state a survival of 76+ but if you backcheck their 76+ you find an aparent clerical error brought forward from here:
I have come across other figures that suggested 250 in total and non cutaneous being about 10% of the total.
More good news on a new drug for graft verses host disease just approved in Canada:
Thanks for posting that, I can't imagine how I missed it. Looks very promising.
Blastic Plasmacytoid Dendritic Cell Neoplasm
My husband came down with what looked like black and blue marks around his hairline in February, 2011. The Dermatologist said it was a skin rash and prescribed a topical cream and shampoo as the scalp was very sensitive.
It faded and came back, all blood tests were normal. This was now May, 2011. In June, 2011, his whole face was black and blue. It was also on his chest, back and arms. I sent him to my dermatologist and he was sent directly to his internist for blood tests.
In July, 2011 his blood work came back and his platelets were at 68 from 400 in May. He was immediately admitted into the hospital and given platelets. His white blood count was always high, 14, the average is 8-10. Nobody knew why, he went to multiple doctors and got no explanation. When he was admitted into the hospital his white count was around 22, then plummeted to 3.
After the bone marrow biopsy, they found that it had entered the brain. Chemo began and lasted 2 weeks, when pneumonia set it to the lungs. It was a bacterial pneumonia and entered the blood stream and turned to sepsis.
Unfortunately, this is not a success story. He passed away on August 18, 2011 at 54 years of age. A strong healthy man of 6'1" and 200 lbs. This is a horrible rapid disease that is very rare, and his chances were slim to none.
I hope that I never have to see anyone go through this again, it has changed my life.
I am sorry to hear of your loss. My 76 year old father was just diagnosed with this lymphoma, although he has no lesions..they found it when doing a routine sinus surgery to remove polyps. Some tissue came back with the BPDCN.
We are just in the beginning stages, as he is having a bone marrow biopsy and PET scan done to stage it. The doctor indicated that chemo and radiation would be in the future.
I am beyond scared and worried.
I am very sorry to hear of your loss. Survival from this entity is very rare, but there are a few of us.
Surviving the treatment for this is a serious ordeal in its own rite. I was 6 foot 5 and 17 stone (238 pounds) of muscle and came out of the hospital after 191 days over a year, 8 rounds of Hypercvad, an autologus transplant and a tbi later, at 12 stone (168 pounds) a skeleton but still 6 foot 5.
I am still struggling with after effects and am aware of the very high possibility of a relapse, but choose not to live in fear and will deal with the future when and if it comes.
Thank you for your post. We had no idea how rare this Leukemia was. We were told by the oncologist that it was very rare and they had only 3 cases before us, two were still alive and one was deceased, but that gave us some hope. His doctor never smiled, which I found very eerie. The doctors could not even pronounce the name of it. But my husband had hope and he was determined to fight this until the end.
Not one time did I let anyone into that hospital room with a negative attitude. We were positive all the way down until the end.
You sure did lose a lot of weight, and so did I, I lost 30 lbs. in total in 5 weeks, while watching this happen. It was so stressful, I lost my appetite. And you are very tall, I am 5'1", but was a bit overweight, but I would not advise anyone to lose weight in this manner.
If my husband had to be taken home with our Lord, I'm glad God did it fast. To watch someone suffer rips your heart out. He was more upset over losing his hair than anything else.
The chemo was working, his spots went away, but it was a hot summer here in New York, and they had the air conditioning pumping, so I put the cardboard serving trays from the cafeteria over the vents, but he was always sweating and pulled his gown off at night. So I wonder if he might of caught a draft.
The Friday before he died, he went on the following Thursday, he called me at 5am, and said the cat was on his bed and was mad at him for not saying goodbye. I had put her to sleep a few weeks earlier, she was 21 years old, had renal failure and was falling down the stairs. But I knew something was definitely wrong, by the way he was speaking on the phone. I wonder now if she came to get him. Some say the one you love most comes for you, and that was his baby.
The antibiotics are only as good as your immunity system, which was as low as an infant. When they wheeled him down to ICU because of the pneumonia, he was more angry about losing his private room with his DVD and sound bar player. I said they are trying to save your life, but he did not want to hear it. He cursed every top doctor all the way down to ICU, including myself, but I just had to let it roll off my back, he wasn't in the right mind frame.
He never made it to the bone marrow transplant, while in ICU being treated for the pneumonia, he turned pitch black. The nurse said there was no oxygen, I said BS, I got the doctor on staff and he said if there was no oxygen, his lips would be blue, so they ordered platelets and his white count came up and the black turned back to purple.
But when they showed me the x-ray of his chest, 70% of the machine was breathing for him, he was only breathing on his own only 30%. He was going down, and going down fast. I spoke with the doctors and thought about this very carefully. One doctor said that if there is no hope, you must let nature take its course. And he was rightfully so.
So I had him transferred down a floor to Palliative Care (Hospice) unit, since I had decided to remove him from life support and didn't want anyone in ICU living to see that, I did not want to scare the living, plus I wanted it private. They took him off of the antibiotics and antifungal medications. We always promised each other that we would not let either one another stay on that machine if there was no hope.
The next day we were got to the hospital to take him off of life support at 11am, but before we arrived, God had beaten us to it and taken him home.
I figured after, that he had no oxygen in the brain and was delusional from the things he was saying a week prior about the cat. That is when I knew something went awry. I thought it was the morphine they were giving him for his backache, which was unheard of. Morphine is for severe pain, not a backache. They should of prescribed a muscle relaxer.
Oldtree, never live in fear, when our number is called, it's time to go home. But do live your life to the fullest, and live it every day while you still have it, and if you’re up to it, go out and have some fun, because we never know if tomorrow may come.
God Bless You
You are always in my prayers
It is possible that they were comforting him by giving the morphine, it was my choice too to feel no pain and also not to be on a machine, I had expected to die. There is no denying that this is a terminal disease and the medical staff know that, how can they proffer the branch of hope when they know that there is none. I would not want to be lied to and I made that very clear. Doctors could say somthing like "you have a 50 50 chance", and in our context that would mean simply you either will die or you wont. It must be very difficult for medical staff and it not a job I envy.
Many things about my ordeal were out of the ordinary and seemed to be cutting my chances down, the disease itself, my rarer symptoms, no sibling match nor match on the european donor database 14,000,000, rare bloodtype, drug reactions, and on and on.
It is an odd thing to still be here as I had prepared to go away and return to dust. the only thing that I was really worried about and I mean the only thing was leaving two young children fatherless.
Different pain killers have different effects on different people and it is a question of finding the right one for what ails you. I had serious boney backpain that morphine didnt touch but IV paracetamol (acetaminophen) worked wonders for.
I also had pain that a morphine pump nor other pain killers didnt touch, with my initial symptoms of swolen lymph glands and intestional blockage, pain beyond imagine.
The chemo drugs and the rest of the drugs and the strange hospital environment and routines make things a bit confusing so I would concentrate on trying to remember your husband as he was before and how he spoke to you before and wipe the rest of it from your mind. There are no more ifs buts or mabys there is just what happened and hindsight is not necessarily a good thing. You can go round and round with that in your head and i know your husband would not want that. I would not and i made sure that as I went through my journey that I would leave nothing but peace behind me.
You did all that you could and I hope you have peace for the rest of your journey.
Yes, I think they were comforting him with the morphine to shut him up. He was not in pain, he had a bad back, but not pain like a cancer patient has.
He had a history of back problems, and with the chemo medication that was red in color and was given intravenously, he got the shivers as a side effect. That is when he said he had a muscle spasm, and boy did he put on a show. That is when the doctor on call prescribed the morphine.
And it only got worse, sitting there watching it was disgusting to say the least. It was like sitting with a junkie. I actually had to go out and complain to the nurse, that is when they toned down the pain medication.
To sit and watch someone you love with their eyes rolled in the back of their head is disturbing, and when it's not needed, I say speak up. You must have someone there in the hospital to speak for you, or they will do nothing, just what is expected of their job. You need someone to fight for your rights.
Your right, I did all I could do, it was out of my hands this time. All other times, I had control, but this time I did not, and that is what bothers me most, that the control of his destiny was out of my hands. I guess I have the survivors guilt and wonder why I was left back and not taken too.
I just wanted to do a quick update. My son is celebrating 2 yrs post transplant in two weeks. There is still no sign of any cancer. Life is good and I am thankful for these blessings each day. The childrens wish foundation gave our family a Disney cruise to the Caribbean in November. The generosity of others is amazing. My three children are quite excited! I am posting this to give others hope!! My son got diagnosed at 15 years old . He was a big boy 6ft3, after a year in the hospital, a failed cord blood transplant. A donor finally cropped up in Europe,he was given a 5% chance of being successful. He did it! He was 95 pounds at the worst. Over 60 blood transfusions between platelets and packed red blood cells. He will be graduating high school in June and plans to go to aviation school next Sept. He has a shirt that his friends made for him that says I KICKED CANCERS ASS!! I guess the bottom line is we were given a death sentence at the beginning, the numbers were horrible, but as this journey went along, I have seen quite a bit of headway with this awful disease. With each success story the Dr.s learn more and more! Our initial recommendation from a well know hospital in the US was pray. We did alot of it and somehow thankfully my son walked away from this disease!
I am delighted to hear of another success story, another one of us, I am now up to 2 years and 4 months post transplant, 28 months of remission if you like, but it is not over yet. The medical staff will not give the all clear for at least 5 years post treatment, and probably not then with this disease. But we cannot live in fear and I do not.
The statistics are dire with this rare disease, 250 have had it, 20% make it to 2 years and 2% to 5 years, but on researching the backing data in that 2011 literature review I found a clerical error in that the survivor of 76 months had passed.
HI OLDTREE,I had my own stem cells returned 1 year past in APRIL and so far its working thank GOD.I still attend the BEATSON HOSPITAL GLASGOW every 3 months for checkups,I also go every month to have blood taken off because of too much iron,due to blood transfusions,I still have terrible muscle cramps and fatigue hopefuly that will subside through time.I never asked too many questions during my treatment or since, I believe I was in GODS hands and still am so I took every day as it came, good or bad, it was"nt all plane sailing as you know but I got on with it,and with the support of my lovely wife and family I am going from strength to strength.I still dont understand a lot about the type of cancer we have I only know its very rare,so its good to hear about other patients and what they have gone through and how they coped. I feel sad when I hear of youngsters being hit with cancer and keep them in my thoughts and prayers.
Good man bob keep it up. I'm on 6 monthlys now.
After 18 months of fatigue I'd had enough and got huge relief from this course, helping me reclaim some more of my life:
For information, contact St James’s Hospital, Dublin, tel: 01-4103457.
Its a study which involves patients 6 months post treatment who are suffering from cronic fatigue.