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Old 11-22-2009, 01:21 AM   #1
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Autoimmune myelofibrosis

Update: Have remained off the plaquenil for 6 months and am noting decrease in PLTcrt, MPV and Plt volume. Now seeing pervasisve PB signs of dysmegakaryopoiesis(PB smear now shows teardrop red blood cells; pervasive large and giant platelet forms, naked megakariocyte nuclei and collagen or reticulin fibers, abnormal netrophils. Diff diagnoses now include macrocytic anemia, primary or secondary autoimmune myelofibrosis, primary MDS with fibrosis, acute and/or chronic idopathic myelofibrosis and idiopathic fibrisis with autoimmune myelofibrosis. Am dreading inevitable and mandatory BM biopsy.

Best case is lifetime regimen of corticosteroid treatment, worst is repreated chemo to normalize blood counts. Anyone else have experience with the autoimmune myelofibroses?

 
Old 12-21-2009, 12:19 AM   #2
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Re: Autoimmune myelofibrosis

I am sorry for the delay in responding. I've never been on plaquenil - i was on cyclosporine. Don't dread the biopsies they are not that bad. I've had four. I'm one year into remission after the cyclosporine knocked me into it. Ask me any quesitons you like. this is a rare disease.

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Old 12-24-2009, 02:03 PM   #3
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Re: Autoimmune myelofibrosis

hello,
my aunt was recently hostpitalized because of extremely low blood count, and for a while a number of doctors were unable to find out what was wrong. After four weeks of test and what not, she was diagnosed with myelofibrosis. Her doctor wanted to start her on thalidomide. The problem is when we went to see another doctor for a second opinion, he said this was not a typical case of myelofibrosis, he said it was autoimmune myelofibrosis and so thalidomide was not the preferred treatment, but cortisone is. Now we have no idea which doctor to listen to and since it's a very rare disease, we're pretty much on our own.

So I'd love to hear about your experiences and insights.

Any comment will be much appreciated.

Thank you.

 
Old 12-25-2009, 06:16 PM   #4
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Re: Autoimmune myelofibrosis

Thanks to all and good luck with your respective situations.

Magritte, the diagnosis of autoimmune myelofibrosis is usually confirmed by bone marrow biopsy. It also requires the presence of some kind of evidence of autoimmune disease (usually a positive autoantibody blood test). Usually there is the presence of excess reticulin fibers in the bone marrow. but little or no splenic or hepatic infiltration of fibers, unlike in the fibrotic stage of chronic idiopathic myelofibrosis.

You would also expect to see lymphocyte aggregates in the bone marrow in the autoimmune form, and a lack of signs of more aggressive involvement of the megakaryocyte line that you might expect in chronic idiopathic myelofibrosis.

Autoimmune myelofibrosis is usually well controlled with corticosteroids (e.g., prednisone) sometimes with the addition of azothioprine or cyclosporine.

Some of the leading research on the autoimmune form comes out of Turkey, BTW. You can find it pretty quickly on Google.

I had a bone marrow biopsy last week and it really wasn't bad at all. I've had leg cramps at night that were worse. Will let you know the results.

 
Old 12-25-2009, 06:25 PM   #5
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Re: Autoimmune myelofibrosis

Quote:
Originally Posted by keiri View Post
I am sorry for the delay in responding. I've never been on plaquenil - i was on cyclosporine. Don't dread the biopsies they are not that bad. I've had four. I'm one year into remission after the cyclosporine knocked me into it. Ask me any quesitons you like. this is a rare disease.
keiri,

How were you diagnosed? Did you have a diagnosis of autoimmune disease before being diagnosed with the autoimmune myelofibrosis, and, if so, what was it? What were the diagnostic findings of the myelofibrosis, and how did your doctor distinguish it from chronic idiopathic or another form of primary or secondary myelofibrosis? Did they treat you with corticosteroids before starting the cyclosporine? And, did you suffer any adverse side effects of the cylclosporine?

Sorry for being so inquisitive, am just very curious and concerned about all of this as you can imagine.

 
Old 12-26-2009, 11:57 AM   #6
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Re: Autoimmune myelofibrosis

Hi Ocelot. I did have several autoimmune illnesses diagnosed prior to the anemia beginning. I became anemic and I went and got blood tests and they came back inconclusive. Then I kept losing blood and I was hospitalized. Prior to all of this, I had hashimoto's thyroiditis, vitiligo, and other issues. I'm not sure what you mean about the diagnostic findings - what specifically? My doctor distinguished it from other forms of secondary because I did not have anything it could be secondary to. He also did heavy metals tests and other types of MPD tests. He conferred with Dr. Tefferi and in their opinions, I had primary myelofibrosis. I saw another doctor at City of Hope who made the same diagnosis and to begin the search for a donor for a BMT.

They couldn't find a donor after a long time of looking, at least, not a donor that had a good chance of success. My doctor decided to try an immunosuppressant since there is some evidence it keeps the red blood count higher longer and because of my history of autoimmune illnesses and due to family history of autoimmune illnesses. My doctor felt at the time that there had been some research that seemed promising. In all honestly, he took a chance that the treatment would slow the progression of the myelofibrosis and that was all that he was expecting. Strangely enough, I had a full recovery on it which was only possible if I had primary autoimmune myelofibrosis. That was confirmed through a follow up visit at the City of Hope.

So basically they only discovered I had that type of myelofibrosis through trying this drug and seeing how I reacted. Due to how rare this disease was, at the time of diagnosis there had only been 7 other cases, I believe, it was not an expected result.

I did take prednisone with the cyclosporine at first.

I did have bad joint pain with the cyclosporine at the higher doses. The pain was extremely rough, severe, and I was on the ground for over a 1/2 an hour when I first went on. The typical treatment is to start with unbelievable high doses and work downwards. The drug is quite toxic but that is the only way to do it and find the highest amount of medication. I had stomach pain as well and was prescribed stomach medication to ease it down.

Feel free to ask me any further questions.

Quote:
Originally Posted by ocelot View Post
keiri,

How were you diagnosed? Did you have a diagnosis of autoimmune disease before being diagnosed with the autoimmune myelofibrosis, and, if so, what was it? What were the diagnostic findings of the myelofibrosis, and how did your doctor distinguish it from chronic idiopathic or another form of primary or secondary myelofibrosis? Did they treat you with corticosteroids before starting the cyclosporine? And, did you suffer any adverse side effects of the cylclosporine?

Sorry for being so inquisitive, am just very curious and concerned about all of this as you can imagine.

Last edited by keiri; 12-26-2009 at 12:05 PM.

 
Old 12-26-2009, 09:31 PM   #7
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Re: Autoimmune myelofibrosis

Keiri,

Thank you for being so thorough and responsive. You are the only person who has helped me with any of the questions I've had (except of course my hem/onc who is nonetheless a very type-A research type and a bit rushed and hard to pin down at times).

AF is extremely rare in the literature as I guess you know. The biggest published study I've found is by a group at USC in 2003. There are very few published cases in which any long-term patient follow-up is reported. You are lucky though that you responded to the immunosuppresives in this way and that apparently you dodged the chronic idiopathic form (which is also very rare), which can be very devastating as I understand it.

I have come up with a few other questions. Are you still on the cyclosporine? I guess you were first diagnosed by bone marrow biopsy. Is that the case?

What were your CBC counts, e.g., MCV, HGB and HCT, when you were first tested, and what was the progress of your anemia prior to treatment with the prednisone and cyclosporine? Did you have any other lab or hematologic manifestations before treatment, e.g., changes in the number or form of your platelets or white blood cells, increased LDH, positive ANAs or antiglobulin tests?

Thanks again. I am trying to compare my experiences with those of patients reported in the literature. Yours is the most informative report I've seen.

 
Old 12-27-2009, 02:32 PM   #8
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Re: Autoimmune myelofibrosis

Hi ocelot,

Yes, I am aware it is rare. We joke I hit the lottery of diseases. I have a copy of that study which was given to me at City of Hope. I was told that there is a chance that the disease would return and I would need to take more immunosuppressants, but that this was unlikely. I was taken off of the transplant list as they did not expect me to ever need the BMT.

I am no longer on cyclosporine. I was taken off it after a year with no change in my blood tests. I was diagnosed by a BMB, as you say. I've had quite a few now - I get one every year and go in every 3 months for blood tests.

I don't recall my counts. I do know when I got the first four transfusions I was at or around 6 hgb. After transfusions, I would continue to drop a hgb point or two every week or two before the treatment. They gave me 18 months to live based on how my body was behaving. I had no other manifestations you describe.



K

Quote:
Originally Posted by ocelot View Post

AF is extremely rare in the literature as I guess you know. The biggest published study I've found is by a group at USC in 2003. There are very few published cases in which any long-term patient follow-up is reported. You are lucky though that you responded to the immunosuppresives in this way and that apparently you dodged the chronic idiopathic form (which is also very rare), which can be very devastating as I understand it.

I have come up with a few other questions. Are you still on the cyclosporine? I guess you were first diagnosed by bone marrow biopsy. Is that the case?

What were your CBC counts, e.g., MCV, HGB and HCT, when you were first tested, and what was the progress of your anemia prior to treatment with the prednisone and cyclosporine? Did you have any other lab or hematologic manifestations before treatment, e.g., changes in the number or form of your platelets or white blood cells, increased LDH, positive ANAs or antiglobulin tests?

Thanks again. I am trying to compare my experiences with those of patients reported in the literature. Yours is the most informative report I've seen.

Last edited by keiri; 12-27-2009 at 02:37 PM.

 
Old 12-28-2009, 08:33 AM   #9
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Re: Autoimmune myelofibrosis

Thanks again, Keiri. Yours is a very reassuring success story. It sounds as if you were very ill and that your doctors did a great job. Congrats.

My biopsy results should be back later this week . . . hopefully they will show minimal problems and my HGB will stabilize. Will let you know.

 
Old 12-29-2009, 09:30 AM   #10
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Re: Autoimmune myelofibrosis

Hello Ocelot and Keiri,

I was very happy to see the detailed feedbacks you've provided. I feel much more informed already.

Keiri,

Iím so happy to hear that youíve recovered, youíve actually erased the rather hopeless picture some of the doctors have shown us.

You both seem to have researched a lot and fully understand the many aspects of this disorder. So I hope you will help me with some of our questions below:
1. Iíve looked for the USC 2003 study and I found one that was published in 2001. If itís not too much trouble could you send me a soft copy if you have one.
2. If it is ok with you to share, could you both write at what age you were diagnosed (Iíve read and heard that MF and Autoimmune MF patients are usually above 60, my aunt is 42)
3. What was the lowest Hgb value you saw in your tests? Ėmy auntís dropped to 4.5 within two weeks of transfusion
4. Where have you been treated Ėjust in case-. Keiri, I understand you were treated at City of Hope, Ocelot what about you? We might be looking into interntional options in terms of doctors/hospitals.
5. Did either of you get corticostreoids as primary treatment Ėbecause thatís what our doctor is suggesting and he also claims to have treated another A MF patient with high doses of corticosteroids and he fully recovered in 6 months-
6. Finally, the trouble weíre starting to have is the aggregation of iron in the body due to transfusions. Doctor says the body will tolerate this until about 20-25th transfusion. Now we are around 8-10.


Thank you very much for sharing your experiences. I realize some of the questions may seem a private or maybe irrelative, but I hope to gather as much info as possible as it is a very rare case.

And Keiri, yes, we seem to have hit the disease lottery as well.

 
Old 12-30-2009, 06:51 AM   #11
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Re: Autoimmune myelofibrosis

1 -- I'm not sure of the exact year. I do know the main writer was someone named Pullerkat and I believe Ocelot has posted it in another list here somewhere. Perhaps he can direct you. I'm in New York for the holidays so I do not have my documentation with me.

2 -- I am 32. I was diagnosed at 30.

3 -- My lowest was 6.1 - that was when I was rushed to the emergency room. After transfusions, I was carefully monitored so when I got around 8 or 9 they would pump me back up again.

4 -- I would stick to Tefferi, Silver, Mesa, and the other well-known US leaders in their field. < edited >

5 -- I had prednisone for a short time but found no positive change there.

6 -- Yes, but there are options for that too such as chelation (sp?) and exjade. Also, that is why they try these things such as immunosuppressants so that the blood counts will stay higher longer. If your aunt is not being treated with anything, I would see someone else, and quickly. My first choice being Tefferi. Where are you located?

Keiri

Last edited by hb-mod; 12-31-2009 at 10:51 AM. Reason: Promoting other forum, providing link

 
Old 01-15-2010, 10:36 PM   #12
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Re: Autoimmune myelofibrosis

Hi Keiri,

My husband has MF (2006 dx). There are questions of auto immune processes. He is transfusion dependent. Transfused blood seems to be destroyed quickly. Do you remember the results of your ANA and Direct Coombs tests?

Many thanks!
Linda

 
Old 01-17-2010, 02:35 PM   #13
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Re: Autoimmune myelofibrosis

No I'm sorry I don't have the results of those tests.

 
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