Confused about my diagnosis

[Leon]

Hi, I am 48 years old with no history of any desease except recurring kidney stones over the past 25 years. Over the course of the past 9 months or so, I started having fluctuating platelet counts, which were found because of pre-op tests for dealing with Kidney stones. Prior to that, I have had no history of any blood disorders. The counts (000s/microLiter) were 246, 80, 213, 120, 120, 139, 62, 46... The last three were while seeing a hemotologist and the later consistent decrease was over the course of 6 weeks. A BMB was done when my count was at 62 and the diagnosis from the pathologist was that it was MDS, though there were no chromosomal abnormalities of any sort and and blasts were at 3%. The hemotologist found it odd that the peripheral blood was actually showing a lower number of platelets than would be indicated by what was observed in the bone marrow. When the count reached 46, she decided to try, partially as a diagnostic, prednisone. After 1 week on 100mg/day the peripheral count went to 110. She decided to drop the dosage to 60mg/day for two weeks, and the count returned into the mid 60s. Again she raised to 100mg/day for 1 week and the count went to 140. Now after yet another week of 100mg/day followed by a week of 80mg/day the count was 111. She is now decreasing the dosage to 70mg for two weeks, presumably with the goal of further reduction in the dosage.

I guess one thing I am confused about is whether I do in fact have MDS or not. If I do, it would have to be in the very low risk zone of the various ways in which MDS is staged, but my understanding was that MDS does not respond to corticosteroids alone. Any ideas, suggestions would be very welcome.

[Leon]

All my other blood types are according to the hemotologist "rock solid"

[Neil Cuadra]

Leon,

Without dysplasia, the diagnosis of myelodysplasia can easily be questioned. However, you mentioned only that no chromosomal abnormalities were observed in your bone marrow biopsy results. Were misshapen cells observed? I suggest asking your hemotologist about this. You can ask about your cellularity too. Also, if you don't mind trying to sort though the medical jargon you could get a copy of the BMB lab report and see firsthand what it says.

The crazy way your platelets fluctuated (246, 80, 213, etc.) made me think, at first, that higher counts after you started prednisone might just be a coincidence of timing, but now that you've been on higher and lower doses a couple of times and had your platelet count go up and down in parallel, the correlation seems to be a true cause and effect. Every patient is unique, so a treatment that's working is like money in the bank.

In a way, treatment is more important than diagnosis, since what to do about your low counts is more important than what they call your condition, but I still encourage you to ask questions until you have more confidence that you have the right diagnosis, because knowing the disease and its subtype brings to the table a wealth of statistics and years of medical research, much of it based on disease categorizations.

[Leon]

Hi Neil,
thank you for the quick reply. I will indeed ask for the detailed print-outs from the pathologist. The Prednisone treatment obviously is not a long term solution given the side effects, so my presumption is that the goal is to achieve a stable count that is high enough to be acceptable in terms of bleeding risk and then hopefully do nothinge else. At least that would be the case for an ITP condition. So I guess the trick is to be able to tell if that is indeed what I have.
The term used by the hemotologist to describe the marrow was a bit colloquial: "the cells responsible for platelet production in the marrow were looking 'haggard'" While the other two lineages looked very good. I have read that MDS and ITP are not completely separate, in that MDS can have some autoimmune aspects to it:
http://online.haematologica.org/thj/2004/6200414a.pdf

[tytd]

Hello Leon,
Thanks for the link to the article. I've been suspicious about a link between ITP and MDS in some patients but had not seen that article. I had what I thought was ITP for greater than 20 years until I finally got a bone marrow biopsy a few years back and was diagnosed with MDS just on basis of dysplasia in the megakaryocytes and a macrocytic anemia. However I will tell you that one pathologist disagreed and did not read a lot of dysplasia so there can certainly be different interpretations of what is considered dysplasia or not. I did not respond to Prednisone taken about 3 years ago for a very brief period.
I would be very wary of the diagnosis of MDS in your case due to the significant fluctuations in your counts. Are you sure that you have not been given any drugs that might affect your platelet counts?? If the diagnosis still seems in question after you taper down off the Prednisone, I would definitely get a pathologist or MDS specialist who has seen a lot of MDS bone marrows to review your bone marrow slides and give you a second opinion.
Also many people can do fine for years without bleeding (with certain preventive cautionary measures) with rather low platelet counts. Of course you would need a higher platelet count if you were undergoing surgery for your kidney stones. Good luck and please let us know how you get on with the Prednisone. Tytd

[annmonster]

Just a thought ..... perhaps a second opinion from another Hematologist might be of value in your case......are there any teaching hospitals or cancer centers near you ???? Feel free to ignore my 2 cents worth, as I'm not a Dr. ...

[Leon]

Yes, there was some initial suspicion about medications I was taking Chlorthalidone and Uloric for kindey stone prevention (not that it seemed to help), but I have been off both for 6 moths, so unlikely relationship at this point. As some of you have recommended I will want to get a second opinion on the MDS. My hemotologist for now has struck it from the diagnosis in favor of ITP, but I do want to find out more detail about why the pathologist seemed to think it was clearly MDS with so little hard evidence in terms of blasts or in terms of cytogenetics, so I am guessing it was the look/morphology or possibly the cellularity of the megakaryocytes that was an indication (http://www.nespath.org/PDF_files/Has...06_lecture.pdf). What does blur all this is the seeming response to Prednisone: http://dissertations.ub.rug.nl/facul...lItemRecord=ON