AA evolving into MDS vs. hypocellular MDS?

[Coping1]

I have posted before, but it has been a while. I have been dealing with other health issues and have tried to put my MDS/bone marrow transplant out of my head for a while but now my 3rd bone marrow biopsy is coming up in Nov and my doctors are putting it in the forefront again.

There has been some disagreement between my hematologist here and the BMT specialist that I am seeing as to whether I have MDS, AA or hypocellular MDS. The 1st biopsy showed "clear dysplasia and dyspoiesis"-the 2nd biopsy showed "10-20% cellular marrow with ringed siderblasts, consistent with myelodysplasia although aplastic anemia evolving could not be ruled out". The Dr. also talked about it being hypocellular MDS.

This 3rd biopsy is suppose to, hopefully, clear up some answers, but I fear it will only create more questions:/

I am confused between the difference of AA evolving into MDS vs. hypocellular MDS.

Can anyone shed any light on this for me? How does the treatment vary?

Thanks in advance for any help you can give me!

~Coping

[Sally C]

Hi Coping,
There are alot more knowledgeable people on the forums than me so all I can give you is my husband's experience with MDS.
It took 6 months for a diagnosis of MDS after finally going to the National Institutes of Health in Bethesda, Md. - 4 months after starting transfusions.
Initially he had hypocellular MDS which was part of what caused the confusion. He then had hypercellular MDS for a while and is now back to hypocellular MDS. Our oncologist is an excellent MDS specialist. On our first visit she drew 3 overlapping circles - one circle for MDS, one for AA, and one for PNH. She explained that sometimes the diseases can overlap. She thought in Don's case it may be a MDS/AA overlap. As I mentioned, he ended up with a diagnosis of MDS. Beyond that I can't offer anything else. These diseases are so complex and changing that I don't know that the doctors always have much of an explanation of things - just a way to try and treat the disease.
I know this doesn't give you much information - just one case study.
I wish you well.
God Bless,
Sally

[Coping1]

Thanks for your reply Sally!

I have had an autoimmune blood disorder for 30 years and have been treated with Cytoxan twice-that is what the Drs. think has damaged my bone marrow.

Every time I get any type of infection it triggers my blood disorder and I wind up in the hospital. My white count is so low, I get infections all the time-plus I have an immune deficiency-so it is kind of a slippery slope.

My Drs. both have a little different take on my situation. My hematologist here feels that the exact diagnosis doesn't really matter, that I will need a BMT whether it is AA, MDS or a combination. The BMT Dr. in Nashville is worried that I will have a flare up of my blood disorder during the transplant process, which could be "disastrous", and wants to take the "wait and see" approach and keep doing biopsies until we determine exactly what type of damage I have.

I do want to be cautious, but in the meantime I keep being hospitalized with sepsis which I know is not good. My hematologist here is the one who has to deal with each hospitalization and she said eventually one of these infections is going to kill me, so she feels more of an urgency. I do take neupogen shots when my WBC gets really low, but my count is not responding to the shots like it used to and I am requiring more and more RBC and platelet transfusions-especially when I have an infection.

Sorry, I am rambling. Like I said before in my earlier post, I had tried to put this out of my mind while I was dealing with other things but now the biopsy date is getting closer so I'm trying to sort it out in my mind again.

What treatments has your husband had, if you don't mind me asking?

[Sally C]

Hi Coping,
You have really been through alot haven't you!
Don received Campath at NIH in April of 2009. He remained very transfusion dependent for a couple of years after that - platelets and red cells. He then went on Cyclosporine for about 9 months and that did nothing.
He was the first in a clinical trial for Promacta (Eltrombopag) starting in March 2011 at NIH and that started things rolling to where he is completely transfusion independent now. They stopped the Promacta when his platelets hit 100,000 due to concern for clots and he has remained at that level and is still off the medication. His reds normalized on the Promacta as well and his whites normalized a while after the Campath.
There is a detailed account of part of his journey - especially the Promacta study - under "Clinical trials". It truly has performed a miracle as he probably had over 125 transfusions before taking that.
As a second opinion or for additional input you may want to contact NIH (The National Institutes of Health in Bethesda, Md.). They are tops in their field and cutting edge.
I see you live in Kentucky. My step-daughter lives in Newport - just across from Cincinnati.
Please let me know if I can be of further help and I wish you well.
God Bless,
Sally

[Neil Cuadra]

Coping1,

Having two experts give you differing recommendations puts you in a tough spot while making a major decision.

Aplastic anemia and hypocellular MDS look very much alike - symptoms of low counts and too few cells in the bone marrow. The lack of cells makes it hard to look for the cell dysplasia that would be strong evidence of MDS. The third biopsy could help with the diagnosis, although with a previous bone marrow biopsy result of "clear dysplasia" and ringed sideroblasts it's unclear to me why they haven't both decided it's MDS. It's true that if you have to have a transplant then it may not matter what disease you have, but the catch is that knowing what disease you have lets you know what alternatives to transplant you have.

MDS can be classified several ways and I think you should know what classification they'd consider you to have. The includes an assessment of the relative level of risk. That's a factor in deciding whether "wait and watch" is worth the risk that things will get worse if you don't act.

Have you considered taking copies of your medical records, including the previous biopsy results, to another doctor or treatment center for a third opinion? We each have our stories, but anecdotes from forum members here may not give you enough information compared with another expert who is looking at your full history. Your unconfirmed diagnosis, long-term autoimmune disorder, and sepsis incidents no doubt make you unique, and that's when expert advice is most valuable.

[Coping1]

Originally Posted by Neil Cuadra

Coping1,

Having two experts give you differing recommendations puts you in a tough spot while making a major decision.

Aplastic anemia and hypocellular MDS look very much alike - symptoms of low counts and too few cells in the bone marrow. The lack of cells makes it hard to look for the cell dysplasia that would be strong evidence of MDS. The third biopsy could help with the diagnosis, although with a previous bone marrow biopsy result of "clear dysplasia" and ringed sideroblasts it's unclear to me why they haven't both decided it's MDS. It's true that if you have to have a transplant then it may not matter what disease you have, but the catch is that knowing what disease you have lets you know what alternatives to transplant you have.

MDS can be classified several ways and I think you should know what classification they'd consider you to have. The includes an assessment of the relative level of risk. That's a factor in deciding whether "wait and watch" is worth the risk that things will get worse if you don't act.

Have you considered taking copies of your medical records, including the previous biopsy results, to another doctor or treatment center for a third opinion? We each have our stories, but anecdotes from forum members here may not give you enough information compared with another expert who is looking at your full history. Your unconfirmed diagnosis, long-term autoimmune disorder, and sepsis incidents no doubt make you unique, and that's when expert advice is most valuable.

Neil,
I have been to Johns Hopkins, Case Western & Cleveland Clinic twice for my other blood disorder/infections. The last visit to Cleveland Clinic was May, 2011 and they are the ones who first brought up MDS and suggested the BMB.

I probably will seek out a 3rd opinion if this 3rd biopsy is inconclusive, but in addition to dealing with this, I have monthly infusions and plasmapheresis for my autoimmune issues and am fighting infections every other week. The thought of starting with another Dr. seems daunting:/

My local hematologist has given me the diagnosis of MDS and the BM specialist I went to told us that in his "heart of hearts" he felt that it was MDS but is afraid that the BMT will be too risky because of my other problems. The only other treatment that has been mentioned is Vidaza, but neither one of them seem too eager to try that.
The Dr. in Nashville, was very upfront and painted a pretty grim picture-we felt kind of shell shocked when we left-but when he called later with the biopsy results he said there were just too many questions left unanswered and their whole team agreed that right now the risks out weighed the benefits and a 3rd biopsy was needed.

Right now my counts are pretty decent (last CBC hgb-8.6, wbc-1700, plt-86,000), so no decision has to be made immediately, but as my Dr. here says "we are always just 1 infection away from a crisis", so I would like to have a plan. They have already tested my brothers to see if they are a match and they are not-I think when I go to Nashville in Nov. they are suppose to have done a preliminary search of the national registry.

I know that it is best to get expert advice, but one can get so much from hearing about others experiences and being able to ask questions as they come up (and explained in terms I understand

Thank you so much for your answers!

~Coping

[Coping1]

Originally Posted by Sally C

Hi Coping,
You have really been through alot haven't you!
Don received Campath at NIH in April of 2009. He remained very transfusion dependent for a couple of years after that - platelets and red cells. He then went on Cyclosporine for about 9 months and that did nothing.
He was the first in a clinical trial for Promacta (Eltrombopag) starting in March 2011 at NIH and that started things rolling to where he is completely transfusion independent now. They stopped the Promacta when his platelets hit 100,000 due to concern for clots and he has remained at that level and is still off the medication. His reds normalized on the Promacta as well and his whites normalized a while after the Campath.
There is a detailed account of part of his journey - especially the Promacta study - under "Clinical trials". It truly has performed a miracle as he probably had over 125 transfusions before taking that.
As a second opinion or for additional input you may want to contact NIH (The National Institutes of Health in Bethesda, Md.). They are tops in their field and cutting edge.
I see you live in Kentucky. My step-daughter lives in Newport - just across from Cincinnati.
Please let me know if I can be of further help and I wish you well.
God Bless,
Sally

Sally,
I live about an hour from Newport-in Louisville. I see you live in VA-my daughter lives in Roanoke-used to live in Richmond. Small world!!

Your husband has been through a lot with this! Although medical issues are not new for me, I am just learning my way through the MDS, AA world-it's a whole new lingo!!

I will read about your husbands journey-Thank you so much for your help!

~Coping

[Sally C]

Hi Coping,
Another coincidence - my husband used to live in Louisville - worked for Travelers Ins. We are about 35 miles south of Richmond now. It truly is a small world.
Keep your chin up and please keep us informed.
God Bless,
Sally