Immunosuppression

[ColoradoJayhawk]

Hello-

I've was diagnosed with AA 1 year ago. I'm a 29 year old male. My doctor thinks that I may have developed AA from a virus, but nothing has been confirmed. I've been seeing the doctors for this condition for almost 1 1/2 years. During this time my white blood cells and platelets have been in the normal range, but my red blood cells have always been below average. They've slowly decreased in the year that I've been seeing the doctor, but nothing has been done due to the risk/benefit factor. I've had 5 bone marrow biopsy's and each one shows no signs of cancer, but instead an extreme lack of cellularity. They started me on a easy steroid in December and my RBC counts went up a little bit. Their thought is that I should now try immunosuppression. I belive it was the rabbit serum that worked best with my blood typing. My concern is that I've read about a lot of the serious side effects. I haven't had to have transfusions at this point, but I'm sick of worrying about all of this. My doctor said he would be okay with me waiting 6 months, but I want to get this behind me. Can anyone who has gone through this treatment please explain a little more of what I may be in for and what to expect? Thanks in advance.

[Connie]

I am wondering if you have considered trying Aranesp or Procrit first. They can help with the red blood cells. This doesn't work for everyone and can have some side effects also. But I used it for about 5+ years with no side effects. Now, I had been treated with ATG also, but I think that maybe you might want to ask your Dr.s about trying Aranesp or Procrit before you try immunosuppression. Worth a try.
Connie

[Lisa V]

CJH, here's a link to another thread about ATG, which should answer some of your questions: http://forums.marrowforums.org/showthread.php?t=217

What I don't understand is why you were diagnosed with AA if your WBCs and Plts are in the normal range. There are other types of anemia that only affect the RBCs, but AA affects all three lines simultaneously.

I'm concerned about whether you're being given the correct information. First off, the choice of horse or rabbit serum doesn't have anything to do with blood type. Usually horse is tried first and if that doesn't work, then rabbit is the next step, but I have talked to people that have had rabbit serum as a first treatment. I guess it may be just a doctor's preference.

At any rate, this treatment is usually used for cases of Severe or Very Severe Aplastic Anemia. SAA is defined as having marrow cellularity of less than 25% and at least two of the following: a neutrophil count of less than half a billion per liter, a platelet count less than 20 billion per liter, a reticulocyte count of less than 20 billion per liter. If the neutrophil count is less than 0.2 billion per liter, then it's VSAA.

You don't say what your Hgb is, but if you haven't required transfusions your AA, if it is AA, is most likely moderate, so I'm wondering why your doctor is suggesting this line of treatment? I have heard that there are some doctors who will treat MAA with immunosuppressants if it looks like it's headed towards SAA, but in many cases the response to these medications is not complete anyway. What I mean is that "success" is defined as becoming transfusion-independant, not necessarily as a return to full normal counts, so if you're already transfusion-independant, there may not be much to be gained from it.

I would ask your doctor a lot of questions before proceding with this, and make sure you've got the right diagnosis. It sounds like overkill to me. It's not that the side effects are not manageable, it's just that what benefit you stand to gain may not justify any of it.

[ColoradoJayhawk]

Thanks for all of your feedback. However, AA is my diagnosis. I've been going to a reputable center here in Colorado (Rocky Mountain Cancer Centers). I just went and got my counts checked again, and it's still primarily my red blood cells that are effected. The typing I was referring to was DR-15. According to my doctor, this typing responds better to the rabbit ATG. Still not sure if I'm going to go through with the treatment, although my doctor is very confident that this will be a success for me. I was denied payment of the Procit by my insurance company as the feel this is experimental and not a cure. Thanks for all of your advice.

[Ruth Cuadra]

There's an amazing variation of symptoms among people diagnosed with aplastic anemia, but Lisa has given the technical specs for a diagnosis of SAA or VSAA. I agree with her that with only your red cells affected, ATG seems like overkill.

Given that the HLA type DR-15 is also associated with anemia due to vitamin B12 deficiency, I wonder if you have had your B12 and folic acid levels tested?

Regards,
Ruth Cuadra

[Marlene]

Hopefully your doctor has checked your serum B12, folate and iron levels as well as your homocystine and MMA. You can have a "low normal" B12 serum level but still have a B12 deficiency. Any low normal result in the presence of symptoms is highly suspect. That's why you need to have your MMA and homocyctine checked. If either is elevated, then I would suspect a deficiency.

It's unfortunate that many doctors only go by B12 serum levels to verify a deficiency.

Have you considered going to NIH or another center focused on the treatment of AA for second opinion? I felt it was necessary when John was first diagnosed so that we could hear the very limited options available. Most docs only settle on one treatment and stick with it. Also, even though our local hematology/oncology clinic is very well repected, none of the docs had ever treated someone with AA since it is so rare.

Your are right that Procrit is not a cure but it can help improve your HGB. Your doctor can check your EPO levels (How much growth factor your kidneys are producing) and if its under 500, then there's a good chance procrit will help your HGB. But if your HGB is at least 12, it's not advisable to do.

[Wendy Beltrami]

This is a pretty good site describing different types of anemia.

With just your reds affected, it does seem strange that immunosuppression would be recommended.

Just curious, were you tested for PNH and Fanconi Anemia?

Wendy/mom to Grant
dx 12/4/98 AA

[ColoradoJayhawk]

Yes, I've been tested for just about everything....PNH and Fanconi Anemia included. I've had 5 biopsies and my cellularity is in the 5 to 10% of a normal person of my age. I've been seeing the doctor for 2 years and my counts have slowly declined.

[Chirley]

Hi, I have also had just about every test known to man. This includes 5 BMBs as well. However, unlike you, I have a low WCC as well. My specialist has agreed to regular blood transfusions with no more diagnostic tests unless my blood tests change dramatically.

This way I can have some routine in my life and no longer have to wait until I become uncomfortable with shortness of breath. He told me that he prefers to keep my Hb above 80 because chronic anaemia can cause heart failure. I have already had heart failure once from pneumonia and it wasn't very pleasant. Lungs already full of yucky stuff and then filling up with fluid on top of it.

My counts were low but fairly stable for the first couple of years but then about 4 years ago they took a nosedive and that was when I became transfusion dependant. I now have 5 units of blood every 5 weeks. I do not qualify for GCSf because my white count, while always low, is very rarely critical.

My diagnosis is : Bone marrow failure of unkown cause, probably evolving MDS. I have made up a new name : RACL (refractory anaemia, chronic leukopenia) describes it perfectly but doesn't sound as nasty.

I have to go now and put my dogs on the treadmill, sometimes I think it would be easier to wrestle a crocodile.

Bye for now

Chirley

[Chirley]

This one is for Marlene, Hi.

I reading your post and wonder why you mentioned homocysteine and I don't know what MMA is.

I am homozygous for MTHFR and despite taking 5mgs folic acid every day, still have a homocysteine level of 20. B12 folate levels are in the high normal range. When I first found out that I had a high homocysteine (30) it was because I donated some blood to a research study, it was before I developed this illness and I was perfectly healthy at the time.

Lesson learnt: don't participate in research studies, they"re bound to find something wrong !!!!

Bye
Chirley

[Marlene]

Hi Chirly,

You are funny....There are some things I don't want to know either...ha!

I'm not familiar with MTHFR so I can't comment on it. However, I do know that some people have problems utilzing folate in the form of most dietary supplements. This is usually do to some genetic errors. When it comes to the "B" vitamins, you really need all of them. They act synergistically. Typically, they say if your MMA is elevated, it means your B12 is not being utilized and if the homocystine is high, it's a folate problem. However, you need more than folate to effect homocystine. And taking folate alone, can mask a b12 problem.

The main three B vitamins for homocystine are B12, folate and B6. There are bio-active or bio-available forms of these which have shown to be more effective. B12....methylcolbalamin form; B6....p-5-p form; and folate.....methlyfolate. You can get a prescription with all three. It's called Metanex. Or you can buy them individually, OTC. The Methlyfolate is hard to come by because the drug companies are trying to control it. There are a couple other vitamins that are involved but I cannot recall them at this point. But again, I don't know how much of this actually applies to your situation.

One of John's doctors stated that your blood serum levels of folate can be elevated but you may not be utilizing it so it stays in your blood and does not get to where it's needed. The bio-available vitamins do not need your body to convert them to usuable forms.

When we were working to restore John's health after his treatment, I had him on 5000mcg of Methly B12, 50 mg of p-5-p B6, 400 mcg folate and a B50 complex to round things out.

Here's a link on B12 deficiency that talks a bit about MMA. http://www.aafp.org/afp/20030301/979.html

I think it's all very complex when it comes to figuring out what's missing or what will help. Even some of the better nutritionists have a difficult time putting all the pieces together. Knowing what's missing, and knowing why and how to correct it is whole another challenge.

Marlene