Castleman’s Blog

I will try to make this short. My father was diagnosed as having Castleman’s in
August through the VA. As of yesterday the VA told my father there is nothing
further they can do for him, he is a 60 yr Vietnam Vetren that was exposed to
Agent Orange. Is there anything we can do, anywhere we can seek treatment? The
VA has perscribed pain killers to manage his pain and now another antibotic.
Every lymphnod is swollen and greatly enlarged. My father also has Type 2 diabetes. They say they do not have the
resources to help. Please can you offer us any advice. We are located in the
Dayton, OH area.

I was diagnosed with mcd in 3-07.
THE IL6 TREATMENTS HAVE SAVED MY LIFE. BUT,WAITING FOR MY DISABILITY HEALTH COVERAGE HAS NOT SAVED OUR BUDGET. MY HUSBAND HAS CHANGED JOBS TO ACCOMODATE 2 TRIPS A MONTH 5 HRS EACH WAY. IS THERE ANY FUNDING AVAILABLE? THIS TREATMENT IS HELPFUL,BUT WE ARE STRUGGLING .
DEBORAH

Katy,

Since Dr Vanrhee saw you in clinic and was your Tx physician, I am going to forward this bac to him. He will be in contact with you directly for a course of action.

Regards,

JIM
CD Executive Director

Dear Jim and Doctors,

I have made previous contact on ICDO website regarding my father who was diagnosed with Castleman Disease multi-centric late last year.

My father has since January of this year received regular transfusions as he is chronically anaemic. His blood count back in January was at around 112-106 lasting around 5 weeks. Since this time he has had several other transfusions as his blood levels keep dropping.

We only just found out that back in January 31 he has had antibodys show up in his blood which currently seem to reject the transfusions he is now having. The antibodies are:ANTi-E-K, AntiJka, Anti-Cw and this now presents another problem in cross-matching appropriate blood to his own.He was recently discharged from hospital at a hb (haemaglobin count) of 76.

I have a detailed discharge summary which outlines my fathers past medical and present history if this would help including diagnosis of: Castleman’s, Anaemia and Pneumonia. He is currently on 50mg of Prendisone daily and insulin to control the high sugar levels.

Whilst in hospital they treated him with 600mg per day (200mg x 3)over 5 days of Octagam (Intravenous Immunoglobulin (IVIG)which kept his hb levels fluctuating between 84-80, as at discharge (9 May 08) he was hb76 which is still very low.

He was given Aranesp Sureclick due to reduced levels of EPO (erythropoietins) in his kidney and is currently on trial with this to help anemia levels.

He is due to go back for Blood tests on Monday 12 May to check his hb. The haematology specialist is due to start Mabthera(Rituximab) but is waiting to check on his current progress.

He has had methyl prednisone to reduce frequency of transfusions but this was not successful (April 08).

It is such a vicious circle he is facing with this chronic anemia. Unfortunately the anti -IL6 drug is not available in Sydney, Australia – I have researched via pharma company the name of one Actemra R which we could possibly apply for on compassionate grounds. The only problem is it takes up to 8 weeks and he may not be accepted for Clinical Trial.

So this leaves us to the current position of what else can be done for my father? Is surgery to remove a large lymph node/s – (his spleen is enlarged) an option, given his low hb?

Is there anything else you can suggest as the haematology specialist who is doing a good job as much as he can as he has not seen Castleman Disease before. There does not appear to be any CD Specialists in Sydney.

Thank you for your help.
Kind regards Kerri

Anabel,

I cannot provide a legal or medical advice and will only offer my opinion based on my previous history with this disease. Let me forward this email to one of our medical staff for an opinion. I will be back in touch.

Kind regards,

Jim Johnston
ICDO Executive Director

Dear Jim,
Sorry its been such a long time between communications. My e-mail is: MIALIPSTICK@SBCGLOBAL.NET
Feel free to contact me directly with any ideas or comments.
Today I was released from the hospital(Aug 17th), but I have a ton of unmet questions. This summer has been awful for me physically! I went in for blood work due to lightheartedness, one bloodshot eye(X3), vomiting & sudden bruising. My primary care DR. admitted me due to a very low platlet count: only 6,000 & I recieved four blood plasma transfusions along with IV steroids, so it went up to 40,000 as of the 15th, but now its dropped back down to 37,000. Now I am to take 80 mg. of oral predisone each am. after I eat.
I am concerned, IS this a case of multicentric castleman’s ? (as I have written my history to your blog in the past (Nov.’06) that my condition seems to be deteriorating slowly.)This 3 month watch, scan & wait game may NOT be in my best interest. Monday’s bone marrow biopsy was good & did not detect a lymphoma or recurrence of Hodgekin’s Disease. Thank-God!!!… but this oncologist now says I’ve developed an ITP and assumes its probably viral & NOT related to CD because that’s a benign condition and wouldn’t effect my blood count. Is he correct?
My major concern is this: for 5 years all I do is take pain pills, CAT/PET scans, then I’m told to watch & wait… (For What?) Is it possible that my lymph nodes have been exhausted & now CD has invaded my blood? Also,
I developed psychiatric problems while using high dosages of steroids (Dexamethosone)in the past, along with swelling of my face and lower legs. So, what alternate recourse is available to me? I realize that I am supposed to be satisfied that I don’t have a cancer or a malignancy, and of course I AM!!! However, I don’t want to be put on a back burner and be mistreated due to ignorance. Please ask Dr. Fritz Van Rhee to delineate the steps of progression for the multicentic type, as I fear my time is growing near to the end. Aside from all the nerve pain and basic complaints, I have chronic pain in my RT.lower abdomen which is alleviate briefly by pain meds.I use compazine in pill & suppository form as needed and try to function in a normal capacity. I return to work full time after Labor Day and am very concerned about the value of time management. My DRS. say my ITP may take up to 10 weeks or more to correct & can even become a chronic problem! Are they following the right course of action or should I seek medical attention from an outside source, like the Arkansas clinic? I would like to know what’s invading my blood & why I have swollen nodes in my body. Since the steroid treatments in the hospital, my nodes have deflated & provided somewhat relief, but we NEED to identify the main cause, not continue on this “quick fix” gamut, which may be masking the true culprit. Please advise me accordingly, as I have the time & availability now to seek attention elsewhere before the summer expires

JAN – REPLY FROM DR VANRHEE (UAMS)

Please use Methyprednisone? 1mg/kg for 5 days prior to transplant and 5 days after transplant. Do not wait. Go for transplant. I have just transplanted two patients with POEMS who have had stunning improvements. Both of them were very, very sick.

Jan wrote:
Hi Jim

I hope you are well. Sounds like you have been busy and on the move!

I am reposting the following message because it appears to have been overlooked…

I contacted you about stem cell transplants for POEMS a couple of weeks back and very much appreciated you accessing the advice of Dr Tricot and Dr van Rhee for me.

At the end of Dr van Rhee’s message he said:

“Finally, in very sick patients a brief period of pre-treatment with drugs can optimize their condition and maximize the chance of success.”

I’m not sure whether I qualify as “very sick” but my condition seems to have deteriorated slightly in the last couple of weeks. My neuropathy is marginally worse (although I can still walk) and I have been having some suffocation sensations when breathing. However, it is difficult to know what’s POEMS and what’s anxiety and being hyper-vigilant about my bodily functions.

I would like to know what kind of drugs he uses for this “pre-treatment” before SCT and for how long so I can pass on the information to my haematologist in Australia.

Regards

Jan
Reply to this

i know alot of these drs are great just by comments left i know at some point i need to put my faith but come my dr saw alot of patients and gave them medicine and helped them asap me he she left me waiting from 11 of 2006 to 1/07 no meds other then cover up of severe pain. why is my question

Hi Jim

I contacted you about stem cell transplants for POEMS a couple of weeks back and very much appreciated you accessing the advice of Dr Tricot and Dr van Rhee for me.

At the end of Dr van Rhee’s message he said:

“Finally, in very sick patients a brief period of pre-treatment with drugs can optimize their condition and maximize the chance of success.”

I’m not sure whether I qualify as “very sick” but my condition seems to have deteriorated slightly in the last couple of weeks. My neuropathy is marginally worse (although I can still walk) and I have been having some suffocation sensations when breathing. However, it is difficult to know what’s POEMS and what’s anxiety and being hyper-vigilant about my bodily functions.

I would like to know what kind of drugs he uses for this “pre-treatment” before SCT and for how long so I can pass on the information to my haematologist in Australia.

Regards

Jan

Jim and doctors,

Thank you so much for this advice.

I am being treated in the haematology department of a large, reputable public hospital in Australia where they do a lot of successful high dose chemo/stemcell transplants for myeloma, lymphoma, etc. However, I will be their first POEMS transplant so they are a little cautious.

My doctor has contacted Dr Dispenzieri at Mayo and she has passed on the protocols and steroid dosage they use.

I have considered travelling to the US for treatment but I think the downsides of the marathon air travel, cost issues, and lack of support systems and familiar environment would outweigh the benefits. I believe the transplant here in Perth, Western Australia, with the benefit of advice from experienced international experts, is my best option.

Thanks again.

JAN – REPLY FROM UAMS, Dr GUIDO TRICOT:

I agree that there is an increased morbidity and mortality in POEMS and such patients should routinely receive steroids. If your major problem is neuropathy, I am afraid that only transplantation will be able to improve?these symptoms. You should have your transplant done in a center with experience with such patients.

Guido Tricot, M.D., Ph.D.
Director of MTRC
(Myeloma Transplant Research Center)
University for Medical Sciences (UAMS)
4301 W Markham
Little Rock AR 72205
(877) 635-7240

TANYA P,

No problem

Regards,

Jim Johnston,
CD Executive Director

STEVE, Reply from ACRC, Dr VanRhee writes:

He should come to UAMS stat. We can avoid transplant with anti -IL6 antibody therapy

Frits van Rhee, MD, PhD, FRCPath, MRCP(UK)
Director of Immunotherapy and Allogeneic Transplantation,
Associate Professor of Medicine
Myeloma Institute for Research and Therapy
University of Arkansas for Medical Sciences
4301 West Markham
Room 416, mail slot 776
Little Rock, AR 72205
Fax: 501-686-6442
Secretary: 501-2961502 ext 1414
Clinic: 501-2961502 ext.

1379
vanrheefrits@uams.edu

Steve,

First off, I need to caveat this email with the statement that I am nit a physician but a patient myself of 15+ years and CD patient advocate. In reviewing your email, the first thing that was glaring was were was the treatment provided and was the clinician a Specialist in Castlemans DIesaes treatment?

The Organization has established “Centers of Excellence” throughout the US that specialize in the treatment and care of Castlemans Disease patients. I recommend that you seek an opinion from a SPecialist who is well-known in the treatment of CD.

As to your initial comment, I will forward your email to our medical staff for review and will have a response w/i 24-48 hrs. They can recommend a treatment plan or facility to work with in controlling this diesaes.

Regards,

Jim Johnston,
Executive Director

NEW CASTLEMANS CLINICAL TRIALS :
Please visit

http://www.clinicaltrials.gov/ct/search;jsessionid=A2AB9402A17B1DD5F6EAA308C9B6AEBB?term=Castleman&submit=Search

for an update and information on the four clinical trials.

Regards,

Jim Johnston,
Executive Director

ok but , i know i can get 2ed opioin but, dont want to hurt dr munshi feelings please get back to me asap reverema123@hotmail.com thank you god bless. but yes or no does castleman in what percentage casuese extream pain .

Tanya,

?Lets wait ans see what response we get from the other physicians in the AM. I will call Munshi’s colleague and see what he can do.

Regards,

JIM

pain 13.. and thats no meds i have gerd so tylenol over counter all i can do ,, and now i practically have to beg for pain releif from munshi. he even sent me to a pain cancer dr maureen lynch. she tried patch fentanyl 25 did do much i never tried such a strong opiod and patch for so she said up it 50 2 patch. and i slept all the time . so i recommended what dr munshi did in the beging hydromorphone ER tabs.. now he has me takeing hydrocodon. (vicodin low dose ) and its not working that well . weres of fast. pain a 12

Tanya,

I have ust sent over 5-6 other referrals. Please advise if any work for you.

Regards,

Jim Johnston, ED

well please ask is colleague first. for a 2ed opinion please ,, thank you

i thank you for getting back soon, is there any doctors in the east cost. also if i have huge lymph after only 1 and removed and spreading does that sound bad , like my dr never told me after 1st apt what cd i had , but on pathology exam biop.. it stated large cells postive and scattered plasma cells. also, i have a few lesion on my liver. it stated accompanying immunohistochemical stains of lymphoid cells for LCA, WHAT IS LCA. it also confused me by statement saying secondary follicles composed of atrophic sppearing germinal centers surrounded by expanded mantel zone. with slight increase in vascularity. basically all stains positive were. 1.plasma cell 2.atrophic secondary follicles for b- cell ? also dendritic reticulum cells and sparse germinal centers and interfollicular area’s for t-cell antigens and mantel zone cells. but stain for bcl was non-contributory. also biopsy of full lymph/ was post.. for occasional large cells (immunoblast) and the immunohistochemical stains done bmc revealed + for scattered plasma cells but (approx 5%) of cellularity . prominate blood vessels seen. then it states clear-cut hyalinized blood vessels with germ centers were not readily apparent (what does this all mean ) is it plasma cell or hylinvasular or it was one lymph so it was the 1st CD then now after removel it spread to plasma cell / mc castlemans. im lost …

Hi I was just diagnosed with plasma cell variant castlemans disease. can anyone please help me. From all the things I have been reading it just sounds like a death sentence. I am only 31. I just want to know about possible treatments. I am the 1st ever diagnosed case here in my city and the doctors are pretty stumped. i am getting a 2nd opinion from UCLA here in the next couple weeks and I think that is just so my doctors can confer with the docs there. If anyone has any information on my strain of castlemans please email me at holliesmom2001@yahoo.com thank you

My husband had virtually no feeling in his legs from the knee down due to neuropathy. Approximately 1 year post stem cell transplant he has recovered the feeling in his legs with the exception of his toes.

I have had CD and POEMS since 1994. I was treated with a high dosage of prednisone (I am currently off prednisone). I’m now being treated with Cyclosporine & Cell Cept with good results (meaning stabilized). My question is, do the people who get the stem cell transplant regain the use of their nerve damage parts. My feet are damaged bad enough that I need AFO’s to walk. Plus, I have had stomach problems since day one. Thank You, Bobby

Wendy,

I will send this off for a medical opinion from one of our medical staff. Will get back to you here as soon as information is available.

Regards,

JIM

Valerie thanks for your reply, you have given me some hope.

Mayo Clinic, Rochester, MN conducted a study of 16 patients with POEMS Syndrome, treated with autologous stem cell transplantation. Two of these patients also had MC Castlemans disease. Both patients (of whom my husband was one) showed remarkable improvement. My husband is 4 1/2 years symtom free. The study was published in the American Blood Journal in October or November 2004.

I have submitted your email for a medical consult.

Regards,
JIM