Slow-moving AA / Pancytopenia, or What?

[Barbara K]

Hi, David. This is my first post here. My husband was diagnosed last February (after a couple of months of testing) with early/mild aplastic anemia, as well as monoclonal gammopathy of undetermined significance (also very mild) and iron deficiency anemia. Initially it seemed that his counts were dropping at each visit, but mostly things seem to be stable. His RBC count went back up to the very bottom of normal once he started iron but never gets any higher than about 4.3 or so, his platelets tend to range from 120-135ish, and his WBC averages around 2.4, with ANC around 1.2 (lowest counts were 2.0 and .9, respectively). His one BMB showed about 25% cellularity but no clear dysplasia. (There were a few things noted like hypolobulation?--but it was referenced as "rare" or "occasional.") Sometimes his blood tests show reactive lymphocytes, toxic granulation, and large platelets. One time he showed a nucleated RBC. At the most recent test, he did not show monoclonal gammopathy but did show hypogammaglobulenemia. They checked for nutritional deficiencies but the only thing that turned up was the low iron. He's on watch and wait.

So I guess we are several years out from knowing if his story will or won't turn out to be kind of like yours. Good luck to you and everyone else fighting these blood disorders.

[lindy]

David, seems you're hanging on well.
All the best.

[David M]

Hello Again,

I just wanted to update "My Story" since it has been a little over a year since I checked in...

Things are really about the same for me. I still have pancytopenia... I just had a couple of CBCs in the last couple of months. Here is a summary of my past 2 CBCs (just to give a flavor of where I am):

==========
4/3/2014:
==========
RBC - 2.95
WBC - 2.64
Lymph # - 0.99
Lymph % - 37.5
Neut. # - 1.18
Neut. % - 44.6
HGB - 10.4
HCT - 30.9
Platelets - 36

============
6/24/2014:
============
RBC - 3.02
WBC - 3.9
Lymph # - 1.17
Lymph % - 29.9
Neut. # - 1.98
Neut. % - 50.6
HGB - 10.6
Platelets - 51

For the last couple of years, my platelets have been hovering in the 35K range... I know platelet counts tend to bounce around some, so I am not getting too excited about the reading of 51K on 6/24/14 -- although it would be nice if that were the beginning of an upward trend!

Still not having much abnormal bruising or bleeding... have not had frequent infections (no more than usual anyway)... and I have reasonable energy -- although I seem to tire out pretty quickly. I just take a break, then get back up and press on... I am not taking any medicines for this or undergoing treatment of any kind at present -- no transfusions to this point. For now, still in "watch and wait" mode.

I would love to hear from any of you out there who have (or have had) a similar slow-moving pancytopenia situation. I think there are several of "us" out there. I'd love to hear from you!

Until next time...
David M

[bailie]

"Slow motion" must be a phrase that you cherish. My numbers (red, whites and platelets) took all of six months to deteriorate.

[Whizbang]

David M,

Great to hear that your numbers are looking better and on an upward trend...

May it continue ad infinitum...

All the best and May God Bless!!!

David V.

[David M]

Hello All,

I have not updated in quite a while, so I'll update "My Story"... The short version is -- not much has changed. I still have pancytopenia... my blood counts are about where they were last year at this time. In fact, I seem to have been in a plateaued state for quite a while now -- which is a good thing.

Here are the totals from my last 3 CBCs (10/7/14, 4/7/15, 6/23/15):
RBC: 2.87...2.63...2.8
WBC: 3.7...3.6...3.7
Lymph #: 1.4...1.6...2.32
Lymph %: 38...45.5...28
Neut #: 1.9...1.5...2.13
Neut %: 51.6...42.3...62
Hgb: 10.3...9.7...10.0
HCT: 30.6...27.6...Not avail
Platelet: 46...48...47

Also, on 10/14/15, my hematologist sent some of my blood to GenOptix Medical Laboratory for a Myeloid Molecular Profile (i.e. Janus Kinase 2 [JAK2] V617F Gene Mutation Assay). The results as stated were: "Pathogenic mutations are NOT DETECTED." And also: "No known pathogenic mutations are detected in the genes tested." I do not pretend to understand all of what is in that report... I could list the various areas that were tested (e.g. RNA Splicing, Epigenetic, Tumor Suppressor, Transcription Factors, Activated Signaling), the gene, exons tested, etc. For each test performed, NO mutations were detected. I may not understand all of this, but I know that is good news!

In the last year or so, I have gotten sick with colds several times, the flu, and other little "ailments" -- maybe a little more than usual. I am not bruising much, and I have not had any bleeding issues. My energy has been pretty reasonable with HGB hovering around 10.0. So, I am very thankful that this pancytopenia is so very slow-moving.

I am still trying to drink a lot of water, exercising as much and as intensely as I feel like I can, and continuing to trust in God in prayer -- and in asking others to pray for me. I am thankful to God for the measure of health that I have.

I am also thankful for MarrowForums. I have learned a lot from visiting here from time to time. I would love to "talk" with any of you who have similar symptoms, or if you just want to talk about what is going on with you. May God bless you all!

David M

[Cheryl C]

I'm in a stable state too David, and not on any treatment apart from the IgG infusions 4-weekly. I thank God every day I wake up well after being so unwell 4 years ago. We're fortunate people! I've decided just to live as though I'm a well person while like you, monitoring my results so I know how I'm going.

[edithr]

David - thank you for thinking of us and keeping us updated. I'm not sure of my tone will come through in this post, but BORING is UNDERRATED! Love, love, love that nothing seems to change for you. I mean that in a good way.

Do they do bone marrow biopsies on you? Who determines how often you should go for bloodwork? Have you considered that this just might be your normal?

[David M]

edithr,

You are so right! In this case, BORING is GREAT!

The last bone marrow biopsy I had was in June of 2009... I have had 3 of them through the years (I had to be sedated for the last two since my bones are apparently hard as rocks!)...

I go in about 3-4 times per year for CBCs. I have a local hematologist who I see twice per year, and I go once per year to a doctor at Vanderbilt for a second opinion on my situation. Sometimes my GP will also do a CBC if the situation calls for it. My visits with these doctors have been mostly "boring" as you say... again, that is a GOOD thing.

Is this my "normal"? Good question... but I don't think so. I have recorded my CBC results in a spreadsheet dating from 2000 (when we first noticed I had a problem). I also had a few of my CBC results I rounded up from other doctors from the early-mid 1990s... if you look at my blood counts over the years, there is a definite gradual downward trend -- most noticeable in the platelets -- but in reds and whites as well. Since 2009, my counts have been reasonably stable... but all still low.

So, maybe things have stabilized at this level? I don't know... I guess there is no way to know except to keep an eye on it -- to "watch and wait."

[KatailS]

Hi David
This is my very first post to this forum
I am on a very similar journey as you are..... only mine has begun much more recently. In November 2014 my routine blood work came back all out of whack. My primary care physician called me the very same day and requested I get retested (to rule out lab error). The repeat tests came back worse (not surprising as they pulled another 4 tubes out of my arm within a 24 hour period!). My CBC showed:
RBC 2.87, WBC 2.6 (with low neuts and lymphs), hemoglobin 10.4, crit 32.1, MCV 112, MCH 36.2, platelets 104
I was otherwise in the best health of my life, having spent the previous two years working out and eating right. As far as I know, my previous blood work (a couple years prior) was 'normal'. My doctor repeated the CBC, chems and added several more tests over the next 3 months but no change and no smoking gun. She was stumped and referred me to a hematologist who then ran a bunch of tests as well. Since then I have done repeat CBC's, blood chems, and bone marrow biopsy and study of the peripheral blood (flow cytometry) which showed a small, 1.3% erythrocyte and 1.3% granulocyte PNH clone. My LDH and haptoglobin remain normal. My doctor has checked my ferritin, serum folate, B12, vitamin D, TSH, reticulocyte, iron binding capacity, hepatitis, immunoglobulin A, M, and G, ANA, globulin, ..... all came back within the normal range.
The findings have not given any real answers. My platelets have fluctuated between 90-68 while all the other values have remained in the same relative ballpark.
My hematologist put me on iron, B12, and D.... and told me to stop drinking alcohol (yes, I miss my wine). I get my blood tested every 3 months (every 6 months for the PNH) and each time hope for better results. I have done everything my doctors have instructed yet my numbers remain as they are.
I came across your post last night and I admit it was somewhat comforting to read your story and to know I am not the only one. I am trying hard not to let this control my ongoing life, but sometimes find it hard because no one around me understands. I feel like a ticking time bomb.... and it frustrates me because I am the type of person who likes to "fix" things. I am open to any and all advice as I am a proactive person.
I appreciate having a place to share, to learn, to feel like I am not alone.
Kathie

[Sally C]

Hi KaitalS,
My husband started transfusions 4 months before he was able to get a diagnosis in 2009. It took a trip to The National Institutes of Health in Bethesda, Md. to finally find out he had MDS - they are the best. The experts in Richmond, Va. (and I mean that sincerely) just couldn't diagnosis. You may want to consider trying to get accepted there. They saved my husband's life - he has been in remission going on 5 years now after over 125 blood/platelet transfusions.
I wish you the best!
Sally

[KatailS]

Thanks for replying, Sally
I am not sure how long to just "wait and see" because for the most part I feel okay.... just not as strong as I used to feel and sometimes more tired and dizzy.... never knowing if any time I don't feel well is because something is lurking or if I am just fighting a small bug, .... or if I am ultra sensitive and behaving like a hypochondriac.
What I can't seem to understand is how my numbers can be off for almost a year and a half yet no one can tell me why. I don't want to just accept that this is my new normal, I want to do something to maintain a healthy body for as many years as I can. And I miss my wine.
I have an appointment with my hematologist on Thursday and am thinking of asking for a referral just to be sure there isn't anything more I should be doing. I don't want to wait and ignore these numbers and, worse case scenario, get sicker to then find out there was something less invasive I should've been doing sooner. Anyone else understand that?

[KatailS]

Originally Posted by Sally C

Hi KaitalS,
My husband started transfusions 4 months before he was able to get a diagnosis in 2009. It took a trip to The National Institutes of Health in Bethesda, Md. to finally find out he had MDS - they are the best. The experts in Richmond, Va. (and I mean that sincerely) just couldn't diagnosis. You may want to consider trying to get accepted there. They saved my husband's life - he has been in remission going on 4 years now after over 125 blood/platelet transfusions.
I wish you the best!
Sally

So glad to hear your husband is doing so well and in remission!

[David M]

Hi Kathie,

Thanks so much for your post! No, you are not alone... there are several of "us" out here battling this -- all with different stories and situations. I have learned a lot from this forum, although it seems not a great number of people have my exact situation (i.e. slow-moving pancytopenia).

When I had my bone marrow biopsies (the last one in June 2009), I was told I had a bone marrow cellularity of between 15-20%. They described me as being "hypo-plastic" or "hypo-cellular" -- in other words, I had less than the expected number of blood-making cells in the marrow for someone my age. The formula for calculating the expected cellularity is "100 minus your age", so for someone my age (currently 52), I ought to have a cellularity of 100-52 = 48% cellularity! But instead, I have ~15% to 20%.

Did they tell you what your cellularity was after your bone-marrow biopsy?

I am supposed to go back to the hematologist later this week, so maybe I can post an update for the year then... but for the most part, things with me have remained about the same in the last year.

David M

[KatailS]

Originally Posted by David M

The formula for calculating the expected cellularity is "100 minus your age", so for someone my age (currently 52), I ought to have a cellularity of 100-52 = 48% cellularity! But instead, I have ~15% to 20%.

Did they tell you what your cellularity was after your bone-marrow biopsy?

David M

Hi David
I really appreciate reading about the different journeys and experiences.... it is helping me to wrap my head around many scenarios. Your experience has helped me to settle down a bit and not be as fearful by having no immediate answers.
I hope all goes well at your next visit
The biopsy report did not use the term "cellularity", but it did say "there are 46% myeloid elements which are well-represented and mature in an orderly fashion". It goes on to say "there are 24% erythroid elements which are megaloblastic and display an expected spectrum of maturation" and "iron stores are markedly decreased to absent"
Another point made is that the "megakaryocytes are normal in number and distribution; they are not as prevalent as one would expect to compensate for the low peripheral count"

No obvious red flags, no definitive causes. But I guess I am pretty "new" to this as well ;-)

[David M]

Well, I guess I am not educated enough at this point to know exactly what all that means. (ha) But there are people on this forum who I know can "interpret" that information. (If no one responds to this post with an explanation, you might post a question to one of the forums and I'm sure someone would explain all of that.)

The good news is, it sounds like things are stable for you at the moment. This gives you a good opportunity to learn about Bone Marrow Failure in its various forms (if you wish) and to think about options going forward. Some people's counts crash overnight and they don't have the luxury of time. Things like finding a good doctor (or doctors) is an important thing to do. Most doctors have little or no experience with this sort of thing -- although they may not volunteer to send you on to a specialist that DOES have experience with it (you may have to take the initiative in this case and find your own specialist/facility you are comfortable with). This is a good time to do that kind of thing.

There are a few of us on the forum that have had many years of "watching and waiting." I wish I didn't have this blood problem -- it is always in the back of my mind -- but I will say that it has helped me appreciate and value life more. Each day truly is a gift!

Feel free to contact me anytime you would like to compare notes, concerns, stories, etc., and I'll tell you what I know... which may not be much. But at least it is good to have someone to talk to who is going thru something similar!

[KatailS]

Thank you David :-)
Yes, every day IS a gift.... and I am learning not to waste any days.

I am certainly open to advice or snippets of knowledge that anyone may care to share. And I will be sure to spend this time to learn as much as I can AND to find doctors I am comfortable with..... just in case. Good advice that I plan to follow.

Wishing you a good visit with your hematologist later this week.... looking forward to a positive report!

[David M]

Hello All,

Well, it is time for a yearly update of "My Story"... Not a lot has has changed in the last year. That is the good news! The bad news also is, not a lot has changed in the last year -- so I still have unexplained pancytopenia.

Here are the totals from my last 3 CBCs (10/14/15, 4/14/16, 6/28/16):
RBC: 2.61...2.81...2.84
WBC: 3.6...4.2...3.2
Lymph #: 1.8...1.7...1.24
Lymph %: 51...39.9...38.3
Neut #: 1.4...1.76...1.44
Neut %: 39.8...41.5...44.4
Hgb: 9.1...10.3...10.3
HCT: 27.7...30.4...Not avail
Platelet: 53...40...44

I have had pretty good energy in the last year -- done some reasonably regular and intense exercise (taking a break recently -- I need to get back on that!). Haven't been sick a lot (a few times -- colds, stomach bugs, and such). Haven't been bleeding or bruising excessively. No swelling in lower legs (well, not much anyway). Still doing the "watch and wait" thing -- not on any meds or treatments at this point, and still not doing any transfusions, etc. Thank God!

My counts started decreasing slowly in 2000 (at least that is when we noticed that they were low), and they continued to slowly decrease until June 2009. Since July 2009, my counts have been "amazingly stable" (as my hematologist at Vanderbilt said yesterday).

I am still trying to drink a lot of water -- more than I ever have in my life. This is something simple that anyone can do, and I think the benefits are numerous. A large percentage of our bodies (and of course, our blood) is made up of water -- so it makes sense to be well-hydrated!

I am thankful for each day of "good health" that God gives me -- I realize this could change at any moment. I do feel sort of "draggy" at times and tired, but I am never sure if that is the low-blood talking or just the way a 52-year old feels. I also feel a little "foggy brained" at times and have a little trouble concentrating -- but maybe this too is normal for a 52 year old? (I don't know, I have never been 52 years old before.)

Thanks for all the great support and info on MarrowForums! It has really helped me to keep some semblance of sanity in dealing with this Pancytopenia / Bone Marrow Failure situation.

David M

[Cheryl C]

Hi David - I'm another stable, 'watch and wait' forum member. After 6 years with chronic idiopathic neutropenia followed by nearly 5 years since my MDS diagnosis, I can agree that it's possible to live a very normal life with lowish blood counts.

Yesterday I hiked 12 km with my son and grandson. Because things can change so suddenly with MDS, whenever I get that kind of opportunity I think, "Maybe this is the last time I'll be able to do this," so I go ahead, even though I know I'll be tired afterwards. How fortunate I am! Like you, I thank God every day that I wake up feeling well.

[KatailS]

Hi David
I admit, I was anxiously awaiting your update to see how you have been doing. I feel as if I can identify with you and your story the most .... and I am glad things are stable and no worse. That is good news! You also have such a great attitude and I find it very inspiring. The are so few people who can truly understand what it is like to have something 'off' and not have an explanation. Being able to rise above it and live life as fully as possible is a gift... you set a very good example.
Keep doing what you are doing....
Wishing you the best

[David M]

Thanks, KatailS, for your nice comments!

I'd be lying if I said I didn't think about my blood condition quite a bit, but I try hard not to let it get me down too much. There are a lot of people out there sicker than I am! I just try to be thankful for the health I do have, and to do the things I know to do that will not hurt but that are positives.

Prayer has been so important as well -- my own prayers, but also the prayers of others who are lifting me up in their prayers.

Thanks again, and if I can ever help, let me know!

David M

[KatailS]

Originally Posted by David M

I'd be lying if I said I didn't think about my blood condition quite a bit, but I try hard not to let it get me down too much. There are a lot of people out there sicker than I am! I just try to be thankful for the health I do have, and to do the things I know to do that will not hurt but that are positives.
David M

I am relieved to hear you say that you think about your blood condition.... even tho you seem so positive and in control. I do as well, not obsessively, but I am still trying to wrap my head around it as best I can while balancing concern, education about is going on inside me and living.... somedays are easier than others.... and it sure makes it easier to know that there are others who are going through similar issues..... and coping and living.... and even feeling "down" on occasion
And BOY do I hear you on the foggy brain part! It drives me NUTS!!!
Do you mind me asking what your MCV and MCH are?

Keep staying positive and keep praying .... and keep living fully

[David M]

I don't mind sharing the MCV and MCH values at all... Honestly, I need to be smarter about all of this! I don't know much about what these mean in the grand scheme of CBCs... but here goes:

MCV: As of 6/28/16, it was 110 fL (normal: 81-98). I went back and looked on some of my oldest CBC reports from years ago. Looks like in the older reports (10-15 years ago), my readings were usually between 98-105. In the last 5 years, it looks like it has stayed in the 105-109 range. Is this slight shift upward significant?

MCH: As of 6/28/16, it was 36.3 pg (normal: 27.0-32.0). In my records, this is the highest reading I've ever had. It usually was between 31-34 in the older reports, and has been 34-36 in the more recent reports. Again, is this gradual shift upward noteworthy?

How does this compare to your MCV and MCH?

So, do you think the "foggy brain" thing is directly caused by having low hemoglobin, etc.? It is hard for me to really put my finger on it. I just know that at times, it is necessary for me to try to learn new things (i.e. on my job), and it seems extra hard to concentrate, focus, and learn -- it is not impossible for me, it just takes much longer than it used to. You are the same age as me -- but you also have low blood counts! So, is it the age, or is it the "fog"? I just don't know. With a HGB of 9-10, is that low enough to induce "brain fog"?

David M

[bailie]

You can include me into the "brain fog" group. I just mailed large envelope to my daughter (w/ extra stamps) and forgot to put the contents into the envelope.

[KatailS]

Originally Posted by David M

MCV: As of 6/28/16, it was 110 fL (normal: 81-98). I went back and looked on some of my oldest CBC reports from years ago. Looks like in the older reports (10-15 years ago), my readings were usually between 98-105. In the last 5 years, it looks like it has stayed in the 105-109 range. Is this slight shift upward significant?

MCH: As of 6/28/16, it was 36.3 pg (normal: 27.0-32.0). In my records, this is the highest reading I've ever had. It usually was between 31-34 in the older reports, and has been 34-36 in the more recent reports. Again, is this gradual shift upward noteworthy?

How does this compare to your MCV and MCH?

So, do you think the "foggy brain" thing is directly caused by having low hemoglobin, etc.? It is hard for me to really put my finger on it. I just know that at times, it is necessary for me to try to learn new things (i.e. on my job), and it seems extra hard to concentrate, focus, and learn -- it is not impossible for me, it just takes much longer than it used to. You are the same age as me -- but you also have low blood counts! So, is it the age, or is it the "fog"? I just don't know. With a HGB of 9-10, is that low enough to induce "brain fog"?

David M

Hi David
Looks like we are in much the same boat there too.... most recent MCV (size of the red blood cell) is 109.2 and the MCH (amount of hemoglobin within each red blood cell) is also higher at 36.5 .... guessing our red cells are trying to compensate for the lack of numbers by being larger? These numbers are calculations so maybe it really doesn't mean much in the big scheme of things....

Are you also on iron and B12 supplements?

The "brain fog" really annoys me. I am willing to blame some of it on age, but I refuse to believe it is all age-related. I am a young 52.... and until this all started I was in excellent shape and health. I do think my pancytopenia is the main cause (or rather whatever is causing my pancytopenia is the main cause)..... I do think it is very telling that you, bailie and I all notice this "fog"....

This might sound like a weird question.... do you ever get the feeling that when you take a deep breath you can't actually fill your lungs all the way? Everyone thinks I am thinking too hard about and that I am going to hyperventilate. It doesn't happen often, but it does happen and it is not a mental thing. I just can't get the full, deep, satisfying breath for a while. I don't know how else to describe it....

Wishing you and your family a Happy 4th of July