Slow-moving AA / Pancytopenia, or What?
Back in May 2000, after a round of sinus surgery, I went in for some allergy testing as part of the post-surgery sinus treatment. As part of the allergy testing, I was sent for a CBC. The allergy doctor called me at work and asked me to come in and re-take the CBC, since many of the results were low. A re-take of the CBC confirmed that my reds, whites and platelets were indeed low -- not dangerously low -- but enough to get everyone's attention. I was sent to a hematologist in Huntsville, AL, and I was put through a battery of tests (including a bone marrow biopsy & aspiration). The end result of all the tests showed that my bone marrow was hypo-cellular (about 25% active cells). I tested negative for MDS, and everything else.
I went in frequently at first for CBCs, and after a while I only went in every 6 months for checks. Through the years, my counts slowly continued to decline. When I first started getting my blood checked, my platelets were around 130K to 150K. Gradually they have declined until a few months ago when they were measured at 41K. Neutrophil counts are down to ~1.3. Hemaglobin has been down to ~9.5. Through the years, I have had 3 bone marrow biopsy & aspirations (the most recent one a few months ago)... still hypo-cellular with ~20% or less active cells. Tests were negative for MDS and PNH.
I was recently sent for a 2nd opinion with a hematologist (BMT specialist) at Vanderbilt. After looking at my test results and running some tests of his own, the doctor at Vanderbilt came to the same conclusions as my local hematologist. They both hesitate to call this AA... it is "unexplained bone marrow failure resulting in pancytopenia" or something like that... but the end-result is the same as AA -- it is just moving in slow motion! I asked the doctor at Vanderbilt what percentage of people with AA have it behave like mine, and he replied, "Almost no one." He said, it does not appear that my pancytopenia is immune-related; it looks more like the bone marrow is just "wearing out."
So, we have been watching this for 9+ years, and I am just now entering the "treatment zone" where treatments are being discussed. If things continue to "progress" as they have, most likely in the next year or so, I will require some type of treatment. Treatment options discussed have included growth factors (temporary measure), ATG, and BMT. ATG does not appear to be a good option for my particular case, since my bone marrow appears to be "wearing out." This leaves us with BMT -- some day... We have already starting to find a suitable match -- my siblings (bro and sis) were not matches... there were 23 preliminary matches in the 13 million member bone marrow registry.
So, at this point, we are in a "watch and wait" mode. My next appointment is not til December 22. This waiting is driving me a little bit crazy, but I am thankful not to have to be undergoing any kind of treatment at this time.
Well, that's my story... Does anyone else out there have (or know of) slow-moving symptoms like this? Is my case of AA, pancytopenia, or whatever it is, really that odd? Any words of wisdom? I'd love to hear from you!
Do you know if they checked your Iron, B12, folate, B6, Copper and zinc blood serum levels? Low normal of these nutrients indicates an insufficiency so find out if they tested these and get a copy of the results. Vitamin D should also be checked. Most doc do not give much credence to nutritional status so you really need to be your own advocate regarding these matters.
Also, you should get a work-up on your hormones.
Did they discuss the issue of "shortened telomeres" with you? The shorter the telomeres on your stem cells means they have shorter life span because don't replicate well. Which means ATG most likely won't work. You may want to pursue another opinion with NIH in Bethesda MD...they would check for that. I think most would have classified your condition as moderate AA by now.
If you are going for non-related BMT, you may want to check out Johns Hopkins. They have recently started a protocol which greatly reduces the severity of graft vs host disease in MUD transplants for refractory AA patients. I think they've had only three or four people go through the process and are having good results. So it is in the beginning stages and they don't have a lot of data.
David, I can appreciate what you are going through as I am going through the same thing with MDS. I have been on the "wait and watch' list thing for over three years now. Since Feb. 2006 I have seen my numbers slowly, oh so slowly drop down, but I am still not at the levels that will trigger any treatment so the Docs tell me. My HGB is just above 10, RBC at about 3, HCT at 31.7. Platelets are not too bad at 140K. Medicare and VA will not autherize treatment unless the Hemaglobin gets down to about 9. Due for a new BMB in October. In the mean time I slug around like a snail and need to rest a lot. Are you having any similar symptoms? Hang in there pal!
David, I'm not sure I agree with the doctor who said "almost no one" has AA that acts like yours. We've heard from people with slow-moving MAA before, both here and on Aplastic Central, but perhaps the particulars of your case are different.
At any rate, I think if I were in your shoes I'd seek out an expert opinion right about now. You need someone who has seen more AA cases than most oncologists or hematologists and who is up on the latest research, such as the shortened telomeres angle that Marlene has mentioned. A few names that come to mind are Dr. Young at the NIH, Dr. Maciejewski at Cleveland Clinic and Dr. Brodsky at Johns Hopkins. I don't have any direct experience with any of them, mind you, but just based on the information shared here I would have confidence in any of their assessments of the situation.
I'm with Lisa about getting an expert opinion, and would like to add Dr Paquette at UCLA as another AA specialist to consider. After my first treatment for Severe AA using ATG in Dec 2006, I relapsed to a moderate form of the disease. Dr P advised using CsA alone, and it has recovered all of my counts (except that my platelets are stuck at 72k). I would think it is safer to treat you now rather than have to go with the more risky ATG/Prednisone approach later. Just my 2 cents..
Thanks for your concern and your recommendations. Yes, I do feel run-down and tired quite often, but I still try to exercise some. Usually I find I can do much more than I feel like doing at the start.
I am looking into the other things that were mentioned in your responses. In fact, I emailed my doctor at Vanderbilt today and asked him about some of these things:
(1) In the testing at Vanderbilt, I was not tested for B6, Copper and zinc, but I was for the other things mentioned. (I have a feeling I was tested for the B6, copper, and zinc with my doctor in Huntsville, but I have been unable to locate those results... yet.)
(2) About the hormone workup: "no work-up on hormones per say-erythropoietin level was high"
(3) He conceded that my case could be slow moving AA or "smoldering" MDS, but the slow course is relatively rare for AA.
(4) He knows Dr. Young and Dr. Brodsky and will discuss my case with them.
(5) Telemore data is in research phases and not routinely done... but perhaps something will come of this in his discussions with Dr. Young and Dr. Brodsky.
Thanks for your help...
I live and receive treatment in Birmingham, AL, just down the interstate from you! I have SAA and was diagnosed in Dec 2006... If you ever do go for ATG or anything, I have posted on this forum about my experiences with it at UAB Hospital... I wish you the best..
Slow progression of AA
Just wanted to let you know that I also had very slowly progressing "Pancytopenia" over a period of 10 years until I was finally diagnosed with aplastic anemia in 2006. My local oncologist watched my blood counts and I had some bone marrow biopsys over the years, but is was watchful waiting until my counts really dropped off. When that time came and I started to experience more symptoms of severe fatigue, I was refered to a specialist at Johns Hopkins, Dr. Brodsky. So there are others like me who have the disease present in this way.
I did finally have a bone marrow transplant at JH using the new protocal and now my bone marrow works right again and I can say that I am completely cured.
Thanks for commenting on my "story." It sounds like your situation is very similar to mine. I have started reading your blog diary from your web site... but I haven't gotten very far (there is a lot there). As I am reading, I keep wondering if I am reading my future (or something close to it)?
I would love to hear more about your earlier history with pancytopenia, and the events leading up to your going to Johns Hopkins. I know that may seem sort of boring compared to the other info, but that is where I am right now in this.
Also, was ATG ever discussed? I don't remember reading that. If it was not used, what was the reasoning for not going that way? Anyway, thanks for your comments, and thanks for your blog. It is very interesting to me.
Hi David and Maggie
My story is the same as yours...I went into have my daughter in 2001, and the cbc before I was to deliver was very low, hgb was 6, platelets 60K. I got epo for 3 months but it didn't help, then was followed by several different hematologists and had many bone marrow biopsies. My lab results all said hypocellular bone marrow or pancytopenia, until getting diagnosed with mild AA with TERC mutation in 2007. In 2009, it progressed to moderate, and I have had 2 blood transfusions. I am currently under Dr. Neal Youngs care, but am not a candidate for transplant, because of the TERC. If you want to talk or need support, feel free to message me. Sincerely, Linda
Linda I am just wondering why you are not a candidate for transplant because of the TERC?
"Moderate" vs. "Mild"?
I have a question for you... Why did they change your status to "Moderate" from what it was before, i.e. what is it that makes one "moderate" as opposed to "mild"?
Also, if you don't mind me asking... how are your counts these days? How slowly are they declining?
I thought I would share my story with you as I am currently without a sound diagnosis as well. I was initially diagnosed with low grade MDS at a local hospital, then switched to AA at a cancer center due to low cellularity. I was treated with ATG (horse) and cyclosporine. It hasn't worked and it has been 4 an a half months and all of my counts continue to be low-WBC 1.9, Hemoglobin-9 (varies)and platelets will drop to below 10 without transfusions. I also have a PNH clone size of 39% and have been taking Soliris since May. I went to see another specialist in New York's Sloan Kettering Memorial Hospital and he said that there are no chromosomal abnormalities or overt dysplasia to clinch the MDS diagnosis and it is not AA because I am no longer hypocellular. My diagnosis is "Acquired Bone Marrow Failure". They feel that there is a lot of "overlap issues" with AA & MDS and it really doesn't matter what you call it--we are just not making blood cells and our marrow is not working. I am soon to be scheduled for a bone marrow/stem cell transplant in the upcoming months from an unrelated donor. I have found peace with this plan because I want a cure and am tired of transfusions. I hope you get the answers you seek and your counts rise as mysteriously as they went down. Just thought you would like to know that you aren't the only one in the world with this diagnosis issue.
Hello David M.
My son also has slow moving AA. He was diagnosed with pancytopenia when he was 3 years old in 2002. It was watch and wait for us as well until his counts continued to decline over the years. He has completed two rounds of ATG, one horse in 2006 and ATG rabbit in 2008. He has had a total of about 6 transfusions during this time frame. His current blood counts are HGB 6.4, WBC 2.4, PLT 32K and his ANC 888. He is scheduled for a bone marrow biopsy & asperation on July 8th at Cooks Children Hospital, Ft. Worth.
A BMT is still up in the air. My son does not have a sibling match and the MUD we have found lives in Europe. His doctors are still not convinced that a BMT is the way to go. We have also seen Dr. Neil Young at NIH and he also states that he didnt think Gage was ready for a BMT. I'm just not sure what that turning point is.
As it turns out the ultimate deciding factor lies with my husband and myself. We are terrified that we may make a wrongh decission. My son Gage is almost 13 and still very young.
I wish you the very best in your journey and my prayers will be with you.
Just Checking In...
Well, I just wanted to check in and report where I am in my "struggle" with this Pancytopenia, or whatever we wish to call it...
Back in September of 2009, I wrote my initial entry in "My Story." It has been right at 2 years since then, and happily, nothing seems to have changed! Things haven't gotten any worse -- but sadly -- they haven't gotten any better either. My platelets have remained at ~40K (recorded as low as 34K), hgb has actually gone up to ~11 (at times), and I feel pretty good. Red cell and white cell counts are in the same neighborhood as 2 years ago as well. I am not bruising or bleeding, and I am not taking any medication (aside from an iron supplement). Also, no transfusions to this point! Trying to guzzle water like never before, and I'm eating better, but still eating my share of junk too... still need to improve in this area!
So, I am "watching and waiting." I go in every 3 months to get checked, and each time I wonder if THIS will be the time when things start to change -- for good or for ill. So far, each time it is the same story -- no change! Some people say the glass is half-empty, and other people say the glass is half-full -- but I'm just thankful to have a glass at all at this point! I am thankful for each new day! I feel good, and have even been able to exercise quite a bit. Kind of a strange existence -- kind of like living on the edge of a cliff -- but on the GOOD edge!
I do not have a clue as to what is going on in my blood. I still wonder "why" at times... Why are my counts low? Why have they trended downward over the last 11 years? Why have they been stable for the last 2 years? Why haven't they improved? Why doesn't anybody seem to have answers about my particular situation? Why? WHY? WHY?????
So, on the one hand, I struggle to try and make sense of it all. And on the other hand, I thank God for each new day. And on the other hand (wait, that is 3 hands, right?), I try not to worry -- but to just leave it in God's capable hand -- I guess that is the 3rd Hand. Perhaps some day, my counts will crash like Maggie's did (see posts by Magpie75)... and at that point I will adjust to whatever that means. But for now, things are pretty good.
Just wanted to check in! Until next time...
Hi David, thanks for the update.
Can understand this waiting & watching phase.
I am also in this mode for the past 2 years.
I see the hematologist once every 3 months for my CBC.
Of course I count myself lucky compare to many others here.
My last visit was last week & my platelet count plunged to 50 (my 'normal' is 100). Doctor said I have an enlarged lymph node on the right side of my neck.
I also had low grade fever for 2 days. Hopefully my platelet count will rebound if its just a viral infection. I have to see the doctor again next week.
Wishing your counts will remain stable for many more years.
Vitamin B12 again
as far i have understood you have got a pancytopenia in all three blood-rows.
Also you don`t have chromosomal changes.
You have checked your Vitamin B12 counts two years ago. Supposedly they were in the range.
If your situation is comparable to this of 12 patients reported in a recent korean study, you could have a 26,66 % chance that your blood problems origin from Vitamin B12 deficiency and will significantly improve after administration of parenteral (i.m.) injections of Vitamin B12 1000ng for seven days, within 14 days.
The patients of the korean study who were sucessful with the vitamin B12 supplementation partly had normal vitamin B12 counts in the beginning of the therapy, which worsened later. Furthermore half of the 12 patients always stayed in the normal range!
As the patients did not have a diagnosis for pernicious anemia, the traditional reason to do a therapy with vitamin B12, in this studyVitamin B12 was administered "to confirm the diagnosis and improve the clinical condition of the patient".
(Vitamin B12 (in the form of cyanocobalamin) is - in Europe - free available in the pharmacy. Also oral intake is useful, and a study has proved that 120 days intake of Vitamin B12 - tablets 2000ng /day give the same resultat as the standard injection - procedure). The whole Vitamin B12-problem seems to be very complex, but regarding hematological changes caused by Vitamin B12 - or - which are "Vitamin B12 - responsive" the supplementation is said to be successfull in more than 90 percent.
I will post the recent study in the MDS-Forum, as both, AA and MDS (and AML etc) could probably get confused with Vitamin B12 deficiency.
Kind regards, Margarete
I read an article some time ago which stated that copper deficiency symptoms with concurrent Vitamin B12 deficiency or prior B12 deficiency are less likely to respond to replacement therapy. It went on to say that people with copper deficiency are more likely to have or had B12 deficiency.
It's becoming more apparent that micronutrients are important in maintaining optimal health.
thank you, this is interesting. I you have got some more indicators how i can find the article again which you mentioned, this would be helpful. But i could search for it by myself later on the day or tomorrow. In practice you will try to do a supplementation, and if it works, it is ok. If not - more reflection is indicated probably leading to a solution (why this copper-VitB12 connection?)
Have you collected any other articles/studies regarding this topic? I have already some, but not many.
I am a 43 year old, athletic mother, like many of you, who has rarely ever been sick. But I want to mention one "unusual" thing, that is that I stopped menstruating December 2008, one month after my 40th birthday. Not sure if this means anything or not.
In 2007, I went in for a routine physical, and my doctor stated that my wbc were low (3.8, red cells are fine around 4.3, plateletes around 150.) Since then, I have been tested for everything and so far it's been negative. I have been monitored by a hematologist who diagnosed me a few years ago with aplastic anemia after a biopsy. (All the numbers took a drastic plunge that day, but 5 days later were back up)
My wbc count is usually between 3.4-2.4, and plt about 150-140, red cells are always fine.
I went to Barnes in St. Louis, and my white cells actually went to the normal range 4.2- first time in several years. Barnes thought that it might have been a lab error with the previous bloodwork because all the numbers went back up so quickly. They looked at slides of the biopsy I had done and said everything looked ok. I went back home and continued to monitor my white cells every 3 months.
For about 8 months they went and stayed in the 5.1 range, platelets around 155 and so I thought everything was fine, and my hematologist was left scratching his head.
Then June 2011 after bloodwork, they began to fall to 3.94, then in September 2.67, and platelets to 125, red cells at 4.51. The hematologist suggested I speak to a bone marrow transplant doctor at Barnes, so I again went back to St. Louis.
The bmt doc said it would be 'medical malpractice' to even think about a transplant at this point and he wasn't sure what to think because I am not sick. So a month later, I had 2 bone marrow biopsies at Barnes and my white cells went up to 4.3 again, and red cells to 141. He said that it appears there has been some injury to my marrow and that it is "cyclical". He told me to go live my life because I am healthy. But when I asked him what he thought it was, he said "aplastic anemia, but not now".
My pathology report says this and I was wondering if anyone would please give me their opinion about this:
Examination of the right and left bone marrow biopsies and aspirate shows hypocellular marrow (cariable <10% to upto 20-30%) with normal maturation in all cell lineages. No overt dysplasia, excess blasts or features of a specific infectious etiology are seen. The histopathologic features may favor aplastic anemia if other secondary causes of bone marrow suppressionn are excluded and cytogentic/FISH studies are normal. Compared to a previous bone marrow study from July 2010, the overall cellularity has marginally improved from 15% previously to upto 30% in current study, however significant core length shows <10% cellularity on the right side. Flow cytometry shows no excess blasts.
Thank you, and thank you for this website! I am a little lost and overwhelmed waiting for the other shoe to drop and wondering when it will.
Pancytopenia Update (Aug 2012)
Well, I haven't posted in a while, so I thought I'd give a quick update of the status of my pancytopenia / AA / whatever. Things are basically the same with me. Platelets have slowly dropped to the low 30K range (at least in the last 3 CBCs over ~3-4 months). HGB is generally ~10. RBC is around 2.8, and WBC is also in that same area.
I am still feeling good, with good energy (able to exercise quite a bit). I have not been bruising abnormally, have not been bleeding, and I have not been getting frequent infections. So, although overall, things counts-wise have gotten a tad worse in the last year, things seem to be going pretty good otherwise. I am taking only iron, Vitamin D, Vitamin B-complex supplements. So at this point, I have not had any real "treatment" or transfusions -- we are still in the "watch and wait" mode... which is fine with me.
I realize that this pancytopenia problem is something that I can't control, but there are some things I can do that I think may be helpful. For example:
(1) I am trying to drink more water than ever. Water is so important to our bodies, and especially to our blood production -- and most of us do not get nearly enough water. So, drinking more water is something easy I can do that will not hurt me!
(2) I am trying to get in as good of shape as I can by exercising -- vigorously and often! I know many of us do not feel like doing much exercise... low HGB will certainly make this difficult at times. But I was told "you can do as much as you feel like doing" -- so, I am pushing myself to do what I can. I have surprised myself in what I can still do! My endurance seems to be less, and I feel more wiped out at times than I should -- but overall, exercise seems to be helpful.
(3) I have tried to have a positive attitude about this whole thing. I have learned to enjoy each precious day of life, since I know this all can change very quickly! My pancytopenia problem has been going on now for over 12 years -- and it doesn't show any signs of going away in my case! If nothing else, I have learned some patience as I am participating in this "slow motion train wreck." Going around moping and complaining is not helpful to myself or to anyone. I've tried to be thankful, instead of griping. Attitude is something that we CAN control. (There is much more I could say here about having a positive attitude, and I would be happy to discuss it with any of you.)
(4) Although I do not go around complaining about my lot in life, I have developed a support network of people who care about my condition. I have asked them to pray for me, and I keep them posted periodically on my condition. This has also helped greatly with my peace of mind.
(5) I have tried to stay informed by monitoring (and occasionally posting to) MarrowForums and reading other related sources. Denying reality is not helpful. The fact is -- I have a rare bone marrow failure problem. It is helpful to be armed with as much information as is reasonably possible as I face the future.
Well, these are some things that I have done that have been helpful. I am still curious about others out there who have had this form of slow (or very slow) moving pancytopenia. I'd love to hear your stories!
If having a good, positive attitude helps fight these bone marrow diseases, you certainly have that going for you.
Great advice and a wonderful outlook on life for all of us.
I wish you the best!
Update - 5/22/13
I haven't updated in a while... so here is an update! :)
All in all, things with me are about the same. Pancytopenia is still in effect, with platelets now down in the mid-30K range. RBC hovers around 2.98-3.0, and WBC is also in the 2.9 - 3.2 range. HGB is in the 10.0 - 11.5 range. Neutrophils are hovering around 1.5.
So, all in all, things are going pretty good. Still no abnormal bleeding, not getting frequent infections, and not bruising abnormally. Also, my energy level is pretty good. I am very thankful, and I hope this lasts -- or improves!
To give you an idea of how slowly my counts are dropping, see the numbers below for my platelet counts. BTW, I am currently 49 years old...
Pre-1999: 179.8 (5 samples)
2000: 121.1 (11 samples)
2001: 99.5 (4 samples)
2002: 93.0 (3 samples)
2003: 82.5 (4 samples)
2004: 85.7 (3 samples)
2005: 67.0 (2 samples)
2006: 65.7 (3 samples)
2007: 58.0 (2 samples)
2008: 54.0 (2 samples)
2009: 44.5 (6 samples)
2010: 39.6 (5 samples)
2011: 45.1 (6 samples)
2012: 35.5 (4 samples)
2013: 37 (2 samples so far)
Definitely a slow downward trend. I have similar (but not as dramatic) drops over this time period in RBC and WBC.
Anybody out there have similar symptoms? I'd love to hear from you...
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