Waiting and wondering

[Cheryl P]

I am a 43 year old, athletic mother, like many of you, who has rarely ever been sick. I have been "creeping" on this site for quite some time but I feel I need to reach out to somebody out there going through something similiar or can give me a little guidance.
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.) 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. 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.

[Greg H]

Hi Cheryl!

Welcome to the vocal portion of the bone marrow warrior club. There are lots of folks smarter than me about aplastic anemia around here, which is good, since they can correct all the stuff I am about to say. (I'm an MDS guy.)

I think you've got some good docs -- too smart to treat stuff they shouldn't be treating. As I understand it, from listening to other folks around here, aplastic anemia is more or less diagnosed through a process of elimination. So, if you have messed up bone marrow, but you don't have leukemia and you don't have MDS (or a variety of other stuff) then you more or less have aplastic anemia.

Your marrow is in fact pretty short on cells, but you don't have blasts and you don't have dysplasia (messed up baby blood cells in the marrow), and, evidently, you don't have chromosomal abnormalities, so that pretty much rules out leukemia and MDS. And your marrow seems healthier in this latest BMB, even if it's still not normal.

It might be that way for the rest of your life. Or your hematologist might be right, and it might cycle for some reason. Or it might, at some point in the future, get worse, and then it would makes sense to treat it.

It's hard to know why your marrow isn't normal now. It could be a genetic abnormality you were born with or acquired. It could be some toxin you were exposed to at some point. It could be some long ago infection that triggered a low-grade autoimmune reaction.

It would make sense to make sure you don't have any vitamin or mineral deficiencies and to correct any you do have. It would make sense to eat a well-balanced diet and keep being athletic.

There may be some other things you can do to help your marrow stay strong. I'm going to leave those suggestions to the AA experts here.

It would be good to not obsess about that shoe dropping. A dozen years ago, when I was about your age, I got a call from my aunt, who told me my Dad (with whom I was not in regular communication) was living five states away with a widow he met at a high school reunion, that he was exhibiting signs of dementia, and that his new girlfriend didn't really want to be his caregiver. I retrieved him form the widow, and my wife and I spent the next five years caring for him. One phone call out of the blue, and our lives were irreversibly changed.

In other words, the shoes are going to drop. You just don't know which shoes. And they often aren't the shoes you happen to be focused on.

But it's great that you are healthy now, that you have joined us here, and that you've spoken up. I look forward to hearing more from you.

Take care!

Greg

[triumphe64]

Cheryl,
Are you somehow asking for a second opinion? These conditions have lots of variations that a different set of eyes might detect.

[Cheryl P]

Greg H.- Thanks for taking the time to write, I really appreciate it.Your advice was so touching and I thank you for sharing the story about your father with me. It is comforting to hear from someone who really knows about bone marrow issues. Although I haven't even experienced a fraction of anything of what people on this website have, this has been devastating to learn.
First, a nurse told me that I had HIV (before I was ever tested and btw...it was negative) because my wbc were chronically low. Then a local hematologist told me I had "something called aplastic anemia, wrote the word on his card, and told me to look it up on google." I left the doctors office thinking, hmmmm....must not be too bad. Then when I googled it, I couldn't believe it.

Triumphe64- Thank you too for responding to my email. I am not sure what I am asking or why I finally wrote in. Maybe it's because I REALLY want someone to say, "someone has made a mistake- this isn't you." Truthfully, I don't even understand what all that medical stuff means that I wrote and it's about me! I just know that for the past 4 1/2 years, I feel like I am living from blood work to blood work to see if something has changed. And at the same time this whole thing seems surreal because I am so very active and never sick.

Who do I talk to about checking about vitamin deficiencies? My primary or my hematologist? I know that when I went to Barnes the first time over a year ago, they said that I had low iron stores. My local hematologist was like, "eh,...that's normal." And is there something I could do to improve or keep my marrow strong?
Thanks for listening to me blather on and on....and Greg H.~your website is great!

[Greg H]

Hey Cheryl!

There are several folks on the forum that are better than I am on supplements and such; I hope one of them will weigh in.

I'm not sure about your doc's dismissal of the low iron stores. I need to know more about that, so I'll do some research and report back. I think you need to have enough iron stores to make red blood cells. But it may be that having a low cell count in your marrow sort of automatically means you have low iron stores. Let me look into that.

Personally, I'd work with my primary instead of my hematologist on the vitamin deficiency think, though your heme should have ordered up B12, folate, copper, and the various iron tests as a matter of course. My primary is the kind of doc who's not afraid to recommend supplements and he does a soup-to-nuts lab workup once a year. That would probably be worthwhile, just to make sure there's not something obvious that everyone is overlooking.

One of the hard things about MDS and AA is that they are rare disease. Docs and even hematologists don't see them everyday. And even all the aspects of how the marrow works, and, especially, what can mess it up, is still being sorted out in the big research institutions. So it can be pretty hard to get answers to some basic questions, like: What can I do to keep my marrow strong?

I hate that your intro to this whole thing went so badly. Most health care folks are great; but some really stink. Sounds like you got a couple of stinkers.

Stuff you don't understand about your labs or bone marrows -- just ask about them here. There are lots of smart folks. I'm going to break down the info you posted about your marrow in a subsequent post a little later to see if that is useful to you.

Take care!

Greg

[triumphe64]

Originally Posted by Greg H

Hey Cheryl!

One of the hard things about MDS and AA is that they are rare disease. Docs and even hematologists don't see them everyday. And even all the aspects of how the marrow works, and, especially, what can mess it up, is still being sorted out in the big research institutions. So it can be pretty hard to get answers to some basic questions, like: What can I do to keep my marrow strong?

I hate that your intro to this whole thing went so badly. Most health care folks are great; but some really stink. Sounds like you got a couple of stinkers.

This is why I asked if you needed a second opinion.

[Cheryl P]

Originally Posted by triumphe64

This is why I asked if you needed a second opinion.

I'm not sure what you mean...a second opinion by a doctor or people on here?
I did in fact have a second opinion, actually a 3rd. My second opinion doc at Barnes said it was not aplastic anemia. But 8 months later my local hematologist said my numbers were dropping and it was. So I went back to Barnes to speak to another doc who said , "your marrow seems to be injured but there's nothing there at this time." But when asked if he were to predict what was happening, he said aa.
Has anyone else ever had it happen like this?

[Greg H]

Hey Cheryl,

I think Triumph is asking whether you should find a bona fide AA expert -- someone who has devoted their practice and research primarily to AA. AA and MDS are both so relatively rare that many folks find it useful to have a consult with one of the leading experts in the field -- particularly if there seems to be some disagreement amongst the docs they are already consulting.

There are a MDS Centers of Excellence, but I'm not sure if there's something similar for AA. The National Institutes of Health in Maryland has some noted AA experts, and a consult there would only cost you for the travel and lodging, if you could obtain an appointment.

It could be that the folks at Barnes are in fact just such experts, in which case you may not need to see anyone else. Has the specialist that you saw at Barnes published on AA or was their an indication that he/she was particularly interested in AA? Transplanters are sometimes more experts in transplant than in any particular bone marrow failure disorder.

Take care!

Greg

[Marlene]

Cheryl,

Your GP can do testing for the major hitters regarding nutrients. You should ask for Vitamin D, B12, Folate, B6, iron, copper and zinc. Ask him to include MMA and homocysteine. These nutrients are needed for blood production and bone marrow health. B12 needs to be in the mid to high normal. Get copies of lab results. You would be surprised at how often they'll tell you they are normal when they are not. Stop your supplements, if you are taking any, about four days before testing.

I would also suggest to check your hormone levels: estrogen, progesterone, testosterone, thyroid (TSH, T3 & T4). Did your period just cease or did you go into early menopause.

Regarding vitamin D testing. If you use Quest Diagnosis, you will have to adjust the result to get a more accurate reading. They use a testing method which has in the past, reported D levels to be higher than they actually are. There's factor you can apply to get the corrected number.

[triumphe64]

Cheryl,
Try Marlene's advice. If there are still problems then you might try Dr. Maciejewski at Cleveland Clinic. I think they will work with you regarding insurance issues if needed. I have been going to him for nearly four years now.

http://www.clevelandclinic.org/staff...ecialty=&cnt=2

[Greg H]

Hey Cheryl!

I promised to help explain some of that stuff in your bone marrow results, so here goes:

"Examination of the right and left bone marrow biopsies and aspirate . . ."

I'm not sure if this pathologist is looking at two bone marrow biopsies separated by time, one from each hip, or if they actually stuck you twice for this one. Even though everyone calls the whole procedure a "biopsy," the biopsy is really the little plug of spongy marrow they pull out of the bone. The aspirate is the liquid portion they extract form the marrow.


". . . shows hypocellular marrow (cariable <10% to upto 20-30%) . . ."

The bone marrow of tiny babies is almost 100% working marrow that makes blood cells. As we get older, some of that working marrow is gradually replaced by fat. The general rule of thumb is that the percentage of working marrow should be about 100 minus your age, or, in your case, about 57%. That's a pretty rough rule of thumb, though, and it doesn't make sense to get all hung up on a few percentage points (or even ten). But, your bones contain only between 10 and 30 percent working, blood-making marrow. So, you are "hypo (means "under" in Greek, therefore "little," "short," "not enough") cellular. You have less than the expected amount of blood-making marrow in your bones. And that's why your docs are thinking aplastic anemia, since one of its hallmarks is hypocellular marrow. Most folks with MDS have hyper or normocellular marrow, though there is a subset of folks who have hypo cellular MDS.


"with normal maturation in all cell lineages."

Despite not having as much marrow as one would expect, the marrow you do have is cranking out all the different types (lineages) of blood cells: reds, whites, and platelets.


"No overt dysplasia,"

Looking at all the various stages of blood cell production in your marrow, the pathologist does not see any messed up (dysplastic) forms. If he/she had seen those, they might think you have MDS (Myelo-dysplasia). While it's not fun to think you might have AA, it would probably be even worse fun to think you had MDS, since that would mean your marrow isn't just thin, but that it's seriously broken in some way.


"[No] excess blasts"

This is really good, because blasts are baby white blood cells, and having an excess of those in your marrow is a possible sign you are on the road to leukemia.


"[No] features of a specific infectious etiology are seen."

AA and Hypo-MDS can both be related to immune disorders, sometimes starting with an infection of some sort. Your pathologist isn't seeing any signs of an infection that would explain the cause (etiology) of your thin bone marrow.


"The histopathologic features may favor aplastic anemia if other secondary causes of bone marrow suppression are excluded"

So, all the stuff we just said about your marrow, unless some other reason shows up, may well mean you have aplastic anemia.


"and cytogentic/FISH studies are normal."

This pathologist was just looking at the marrow and not doing either of the special studies of chromosomes in your marrow blood cells, calls cytogenetics and FISH. These are two different ways of looking at your chromosomes to see if you have any abnormalities. If you did, then you might have MDS. Do you know if they looked at your chromosomes? If they did, and you have a copy of the report, it would likely be in a section labelled "Cytogenetics" or "karyotype."


"Compared to a previous bone marrow study from July 2010, the overall cellularity has marginally improved from 15% previously to up to 30% in current study, however significant core length shows <10% cellularity on the right side."

So, your marrow in the most recent biopsy looks better than it did in 2010, because you have about twice as much working marrow now as you did then. But he/she notes that the solid piece of marrow from your right side is only about 10 percent cellular, so that improvement must be on the other side.


"Flow cytometry shows no excess blasts."

Flow cytometry is another high-tech way of taking a look at all the kinds of cells they found in your marrow and seeing what percentage of each type you have. Here too, they found no excess baby white blood cells, which is, gavin, good news.


I hope that demystifies a little bit at least what the words mean.

Take care!

Greg

[Hopeful]

Hi CherylP,

I think everyone is giving you great advice here.

I just wanted to add that you can have an aplastic crisis but not have aplastic anemia. Bad viruses, surgery, etc. can cause an aplastic crisis. It is possible for the bone marrow to recover from these events. Perhaps this is what you have as your bone marrow does seem to be recovering. Low normal counts are still normal. Can you recall any bad illness or surgery that you had 2 months before your aplastic crisis?

The cessation of your menstrual cycle is sign that something is amiss. You mentioned that your iron stores are low, and this is something that you should get addressed with your primary care. As mentioned, you should consult with a nutritionalist to check for any other vitamin/mineral deficiencies.

As a point of similarity, I had a few sporatic blood tests done before my diagnosis. They were usually done when I was unusually sick or tired, as I didn't have regular blood tests. In 1998, all my counts were down as I battled a major illness. I eventually got better and didn't bother to have my counts retested. In 2003, I had a child. In 2005, I was unusally tired after recovering from pneumonia and my counts were like yours - low normal platelets, good Hgb, low WBC. I got sick frequently after that, possibly because my WBC were lower than normal (although I never had them rechecked). When I wasn't sick, I felt great. In 2008, I got a really bad virus and 2 months later my marrow failed.

If you ever notice unusual bruising or feel unusually tired, have your counts checked. The fact that you are aware that your marrow may be weakened is a tremendous advantage, as minimizing the delay to diagnosis/treatment minimizes the damage to your marrow.

Stay healthy!

[Lisa Z]

I would just say that you should make sure you are working with a bone marrow failure expert - just in case. Most of our "local" hematologists are not specialized in these diseases enough to make important decisions.
But it does sound like you should
"live your life" right now, but keep tabs on things.

[triumphe64]

Originally Posted by Lisa Z

I would just say that you should make sure you are working with a bone marrow failure expert - just in case. Most of our "local" hematologists are not specialized in these diseases enough to make important decisions.
But it does sound like you should
"live your life" right now, but keep tabs on things.

I think this is a good summary of what we are saying. I looked at the roster of doctors at Barnes. If you want to stick with them, it looks like Dr Blinder in St Louis might be a good choice. I based this upon his interests and his publications.
http://www.barnesjewish.org/physicia...?physician=102

[Cheryl P]

I am blown away at all your helpful and caring responses! Thank you!!!!! I am so grateful to all of you and thankful that I finally wrote in in the forums!

Greg H.- Wow! I read your translation to my husband and he said, "That is what we wanted someone (as in a doc) to do ...to tell us exactly what this means!" Thank you so much.

Marlene-I will definitely get my nutrients checked asap!

Triumphe64- Thank you for taking the time to also research. Actually the first trip to Barnes (a year ago) was with Dr. Blinder and we loved him. My local hematologist this year suggested I go to a Transplant doc because he stated, "you might want to go ahead and get a transplant with your age before you become too sick to handle one." He also told me that with my age my survival rate is decreasing. YIKES!

I never saw it before, probably because I am so clueless to all this, but ALL of your advice about seeing a hematologist instead of a transplant doc makes all the sense in the world. You guys have totally given me hope and courage to get proactive here. Cannot thank you all enough, and thank the administrators of this website.
I am sure I will be bugging you all for all your advice and experience!

[Cheryl P]

Just want to report that I went back to my local hemotologist and my wbc were 3.93, my platelts were 155.
I brought in a piece of paper with all the nutrient tests that Marlene suggested. My zinc/plasma 70 (60-130 normal), copper 115 (70-175), HOMOCYSTEINE 7.1 (4-15.4) and METHYLMALONIC ACID 86 (87-318 normal)
I was NOT fasting from supplements when he told me to go ahead and we'll check these out. I called the nurse after receiving results and said I thought I should have been fasting from supplements and she said she spoke with him and he said no. And that he was not at all worried about the MMA result being only 1 from normal range.
Should I ask my primary care physician or just let this go?

[Marlene]

Hi Cheryl,

Since the MMA and Homocysteine were not elevated, that indicates you are most likely OK on the B12 and folate. These two test are not definitive but can help detect a hidden problem that has not yet shown up. From what I understand, the lower MMA is not significant. Do you know what your B12 level was?

Should you retest? If your B12 was low normal, it may be a good idea to do so but it doesn't sound like you need to at this point. How much B12 are you taking?