Does this sound like pure red cell aplasia or something else?

[Lbrown]

Hi there,

I was diagnosed by a hematologist last Jan with PRCA. She did a bone marrow biopsy but they were unable to get any aspirate, so they just went by the "core sample" (not sure what that is called). Her whole explanation is based on suppressor T cells in the marrow suppressing my production of red and white cells, but the BMB could not get a immunophenotype(?) on them.

I tried cyclosporin for 7 months. I mostly took 450mg a day and felt sick every time I took it and had lots of side effects. I don't want to take prednisone. She has mentioned chemo as the next treatment (cytoxan I think?), or ATG, but neither of those treatments looks very appealing.

My HGB is dropping 10 points a week and I have a low WBC. The other week it was 0.9 with ANC of 0.4, but last week my WBC went to 1.3 with ANC of 0.5. I am going for transfusions of red cells about every 2 weeks. I also had agranulocytosis 18 yrs ago and was hospitalized for 2 months with a fever of unknown origin which eventually went away with cyclosporin (I had 2 courses of G-CSF which did nothing).

I am on a new experimental treatment for autoimmune diseases and am being followed by 3 other doctors. She doesn't know about this treatment yet because I haven't seen her in awhile. So far my blood work has been pretty abysmal, but I feel pretty good.

I have a question - my retic count is right in the middle of normal, so I am producing them, and their hemoglobin is high (I guess that indicates I don't have iron deficiency). Its very confusing.

Thanks.

[Marlene]

Are your other medicines for another autoimmune condition and are they also immuno-supressants?

If other than red cells are effected then I don't think it's PRCA. But it sounds like you have other issues that could be contributing to this.

Regarding the retic count. You need to look at the ARETIC, absolute reticulocyte count to see what you are really producing. You can have a normal retic % but it needs to be adjusted for the anemia. The ARETIC is better indicator. Usually, a high normal/above normal is seen when your body is producing lots of red cell when your body is recovering from blood loss or after a treatments that suppress bone marrow (as long as the BM is working properly)

If you have multiple autoimmune issues and your doc wants to treat your bone marrow problem, then I would look into High Dose Cytoxan at Johns Hopkins. It is and has been used for other autoimmune diseases other than SAA and may be able to treat your other issues as well.

[Lbrown]

I *think* but am not absolutely sure, that the retic count was the absolute number. The normal range was 25 - 100. The measurement was 10^9/L, and the number was 48.8. So normal, but it should have been high based on my anemia I think.

The other treatment I am on is high dose ARB - it does not suppress the immune system and I feel pretty good on it, it just hasn't had an effect on the blood counts yet. They've had a good success rate with other diseases such as sarcoidosis (>80%). My dad and 2 cousins had sarcoidosis. I don't have that, but I know if I don't try that treatment I will always wonder what if I had, and I have a couple of doctors who said I am absolutely doing the right thing. So if that doesn't work, then I will have to look into something more conventional.

I'm in Canada so I don't think going to the US for treatment is an option, but I am sure the same treatments are available here.

Has anyone's doctor mentioned their bone marrow failure is caused by suppressor T cells?

[Marlene]

That sounds like it is the ARETIC. If my memory is correct on this, when someone with SAA has an ARETIC at 50 and above, it is one of the criteria they look at to classify SAA and above 50, you are no longer consider to be in the severe category of AA. John's aretic is in the 70 range and that's with a HGB of 11.5 which is still low but livable.

High Dose Cytoxan is not done by very many docs in the USA. But you can always have your doc call Hopkins to consult.