aa or mds?

[carolyn]

Hi everyone,

I'm new to this site. I was diagnosed with aa in 1999 when I was 14, i'm now 24. In 1999 I had recurrent tonsilitis treated with courses of antibiotics and was found to be 'mildly pancytopenic'. I went on to have a BMB aspirate and trephine which showed an increase in megakaryocytes but an overall hypocellular trephine for my age. Antigranulocyte and antiplatelet antibodies were negative, chromosone and immunological studies were described as inconclusive. I have copied this off an old letter - i don't fully understand what it really means, even after ten yrs. When I was younger I never worried about the AA, my mum never made a big deal of it and the heamotologists I saw always told me my counts were ok and to try and lead a normal life.

In 2007 I got a bad flu while skiing and on my return to London was admitted to hospital with neutropenic sepsis, my ANC was 0.8, dropped to 0.5 and I was in hospital for a week - they allowed me to go hom when my ANC got up to 0.9. I was'nt given any treatment apart from antibiotics. Ever since then I've become increasingly worried about the AA.

My original diagnosis was this - Myeloplastic Anaemia with possible myelodyplastic features. I am still not on an treatment, I have my counts monitored by Heamotologist every 3-6 months depending on how good they are.

My counts at best are HB 13, Neutrophils, 2.5, Platelets, 126, reticulocytes 14 they tend to fluctuate between this and HB 13, Neturophils 1, Platelets 100. They sort of go up and down on their own. I had a baby girl last August, and responded well to pregnancy my counts were 'normal' throughout it was great! Towards the last 2 weeks of pregnancy my platelets dropped to 80 bit then went back up to 100 on the day of delivery.

Now that I'm a mum I think more and more about the AA as I really want to stick around to watch my daughter grow up! The diagnosis of possible myelodyplastic features scares me. I havnt had a BMB since 1999 because the doctors say theres no need unless my counts drop. Has anyone had any experience with this sort of problem? Or can anyone help explain to me what the original findings from my BMB (above) mean?

Thanks so much.

Carolyn

[squirrellypoo]

Hi Carolyn.

I'm afraid I don't know enough about either disease beyond my own experiences of having both so I can't really help out with your questions, but I'm excited to finally see another Londoner on here! I'm currently at Kings down in Denmark Hill midway through a bone marrow transplant that's been an absolute breeze thus far... which hospital are you being treated at? I'd been seeing Dr Radia at St Thomas's and also Prof Mufti (MDS specialist) and Prof Marsh (AA specialist) here at Kings and have been ridiculously happy with all three.

melissa

[carolyn]

Hi Melissa,

Thanks for your reply. I am currently seeing Dr Raj at Guy's Hospital in London Bridge, i have heard lots of very good things about Prof Marsh so sounds like you are in great hands! I wish you all the very best with your BMT

Carolyn

[squirrellypoo]

How funny, I could walk to Guy's from where I live! Though probably not for a while now...

I never did quite understand why I was referred to St Thomas's since their haematology department is so much smaller than Guy's, but I've heard rumours they're going to merge into one at Guy's eventually. I sure hope so, as the followup appointments there would be so much more convenient for me!

[Ruth Cuadra]

Hi, Carolyn.

I'm glad that you and Melissa have found each other here. We know that connecting with other patients who understand what you're dealing with can be very helpful. One of you could start a thread for Londoners in the Your Local Area forum, and see who else shows up!

It's understandable that you still have worries about your slightly low blood counts, and the unresolved question of a lingering illness. Doctors generally don't want to do BMBs unless there is evidence that something is changing. From my experience, I know that you might not actually feel dropping blood counts if you are busy with family, etc. so you need to keep up with having your counts checked regularly.

Regarding the information you provided from your 1999 BMB, it sounds like "Myeloplastic Anaemia" was probably the best diagnosis they could give you. Your anemia was evident from your low blood counts and the BMB indicated other elements that are characteristic of myelodysplasia. Here's a layperson's interpretation of your old report:

"mildly pancytopenic" means that all of your blood counts (red, white, platelets) were lower than average but not extremely low. Some people have naturally lower counts and live that way asymptomatically, but it can also be a sign of a bone marrow failure disease.

"an increase in megakaryocytes" -- megakaryocytes are the cells that mature into platelets. An increased production of these cells in the marrow combined with low platelet counts in the blood are often seen in bone marrow diseases.

"hypocellular trephine" -- Trephine is the word for the bone sample taken during the BMB. From this they studied your overall cell counts (cellularity) and found you to have fewer than the normal number of cells (you were "hypocellular"). A rule of thumb for bone marrow cellularity is that it should be about 100 minus your age. At age 14, your cellularity should have been 85-90%. Anything significantly lower would be characterized as hypocellular, which is characteristic of aplastic anemia. There is also a hypocellular form of MDS in which the bone marrow is essentially empty but the cells that do exists are dysplastic, that is badly shaped or the wrong size.

"antigranulocyte and antiplatelet antibodies were negative" -- this means that you didn't have any antibodies that were fighting your white blood cells (granulocytes) or platelets.

inconclusive "chromosome and immunological studies" -- chromosome studies are the key to distinguishing aplastic anemia from MDS. It's good that they didn't spot any clear abnormalities. The results may have been deemed inconclusive because of the hypocellularity -- not enough cells to get a good sample. Likewise, it seems that they couldn't determine if your immune system was the cause of your low counts. If you were to have another BMB, you might be able to get a more definitive diagnosis but there are many patients who remain in the gray area between the two diseases. The importance of knowing the difference is to decide, when the time comes, on the treatment options.

Whatever condition you have must be very stable since you've gone 10 years, and through pregnancy and childbirth, with depressed but not extremely low counts. I'm sure it's comforting to know that aplastic anemia is almost always acquired, not inherited.

Regards,
Ruth

[carolyn]

Thanks Ruth, its nice to have an explanation in simpler terms. So is Myeloplastic Anaemia somewhere between the two diseases or is it a different disease? The reason I ask this is because none of the Doctors have ever mentioned anything about MDS to me and have always reffered to my condition as Aplastic Anaemia...its only when I saw the old notes that I started to wonder about MDS as I saw my cells had 'possible myelodyplastic features.' It is very comforting to know that AA/MDS is not inherited and its nice to see that people like you and Mellissa have had both diseases and are doing so well!

Carolyn

[carolyn]

Hi Melissa,

I used to be able to walk to Guys from my place too, lived just off Bermondsey Street but we moved just before my daughter was born as our flat was way too small for three of us!
Is your BMT from a sibling donor or one from the register? I'm so glad its going well for you. Yes, I did hear that St. Thomas' and Guys are merging together too, that will be good!

Carolyn

[squirrellypoo]

How funny, your MDS even sounds like mine - I had so few cells in my marrow that it was difficult for them to further categorise me so I was just left with AA and "hypoplastic MDS" as my diagnosis. I see both Professors equally in my clinic appointments, it seems to be just a matter of who's available.

And it's even funnier how local you were to me!! I live on a boat down by Tower Bridge, just near the Design Museum, so Bermondsey Street is very close. Small world!

My brother wasn't a good match for me, so I had an Matched Unrelated Donor (MUD), who turned out to be a 28 year old man from America, so now I've got his blood type, too! The good news about having such patheticly low counts and empty marrow is that it didn't take very much chemo (and zero radiation) to kill off what I did have, so I had what's called a "mini transplant", or Reduced Intensity Conditioning (RIC). I credit that (and being young and having less than a year of treatment leading up to the transplant) with having such an easy ride thus far. I came home yesterday, exactly 3 weeks since I was admitted, but there's more on that in the Transplant subforum if you're curious.