Spouse of Pancytopenia Patient

[Barbara K]

Hi, Everyone. I've been mostly lurking on this site for a couple of years now and made a couple of posts but haven't officially introduced myself. So, hello!

My now-50 year old spouse was diagnosed with suspected early/mild AA during the winter of 2011-12, along with iron deficiency and monoclonal gammopathy of IGM. Only his ANC hovered in the moderate neutropenia range (around 900); his red blood cell count improved to almost low normal with iron supplementation (it was about 3.7 upon diagnosis and now is more like 4.3); and his platelets hover around 120-130. So he has been on watch and wait, and we've gone on pretty much as normal. The monoclonal gammopathy disappeared by last summer, which was a relief. But one of his tests did come back saying that hypogammaglobulenemia was present.

In his most recent testing, his red counts and platelets remained just below low normal, but he had large platelets (a frequent finding) and two nucleated red blood cells. This is the second time nucleated red blood cells have appeared (he had one a year ago). His white cell count was 1.9 and his ANC was 940. He showed toxic granulation, which has shown up pretty regularly before, but also Dohle bodies and nuclear vacuolation, which were new. His hematologist scheduled him to return for updated labs in three months and said she might want to do another BMB pending what the peripheral blood shows. His first and so far only BMB two years ago showed 25% cellularity and no significant dysplasia. Because I know his hematologist likely wouldn't be talking about doing another BMB if she thought everything was still stable, I'm finding it hard not to start googling the lab findings and reading random web sites that pop up. If anyone has experience with these kinds of lab findings, I'd be grateful for any words of advice or wisdom! Are they just signs of bone marrow under stress?

So, that's our story. I really admire how supportive you all are of one another and wish everyone the best in your journeys. Thanks!

[Cheryl C]

Hi Barbara K

As you will see by my signature block I have MDS and hypogammaglobulinaemia and have been having gamma globulin infusions since September 2011 (currently having a 2 month break). The infusions have made a big difference to my well-being.

I had abnormal blood results - especially with white cells/neuts/lymphs - for at least 6 years before being diagnosed with MDS. I had a BMB/T in 2008 which didn't show MDS, but my specialist thinks that I had developed MDS at least 2 years before the biopsy which did show MDS and hypogammaglobulinaemia (Sep 2011) so it must have developed around 2009.

I have mentioned this so that you will not hesitate to have a biopsy if advised to do so. In fact you should even request one if you are concerned or go to a different specialist if necessary.

In my own experience GPs and specialists can become complacent when they get used to seeing your pathology results and you are not looking or feeling drastically unwell. I was only diagnosed after I had been ill with bacterial bronchitis and decided to take advantage of a voluntary medical check in my workplace. This GP noticed that my platelets were also lower than normal and insisted that I see a specialist again. In the mean time my previous specialist had retired and the new one followed up with a BMB/T.

Hope this helps!

[Barbara K]

Hi, Cheryl C

It does help--a lot! Thanks so much for your reply. I had noticed your signature before because hypogammaglobulinaemia doesn't pop up much here. And the whole issue of why his white counts hover so much more below normal than his RBC and platelets has definitely bothered me if the diagnosis is mild AA. (And while toxic granulation seems to occur w/ AA, NRBC and large platelets don't seem overly associated with it . . .).

We have, then, wondered if the diagnosis might be on the way to changing to MDS or something else. Mostly we don't notice anything too worrisome symptom-wise; he definitely craves a nap each afternoon and has had more than what used to be his normal share of colds and respiratory infections. To me it seems like he is constantly clearing his throat. He hematologist says that some people do fine w/ a 900 ANC count if the neutrophils they do have are like the Green Berets of neutrophils!

We're at a large hospital in the midwest associated w/ a Big Ten university. We like his doctor but have agreed that pending what the lab results show after this next visit and what she recommends, we will seek out a second opinion at a place like Mayo. A while back she had mentioned that she could give him some sort of shot to boost his ANC count if he wanted, but he turned it down. I don't know if that was what you are doing or something different. Can I ask how you feel different--fewer illnesses? Less fatigue?

Or, wait! I'll go back later today and read your old posts to catch up on your story before I ask you to fill in more details. Thanks so much for taking the time to respond. And best wishes for your own continued well-being. I'll keep you in my thoughts.

[Cheryl C]

Hi Barbara - I have had a lot of help from this site, so it's great to be able to encourage someone else.

I believe the gamma globulin infusions boost my immune system. They don't raise my white cells/neuts/lymphs but I'm sure they help me to avoid infections. See http://www.utoronto.ca/kids/Hypogam.htm for more info on hypogammaglobulinaemia. Also I don't get as tired or have as many aches and pains (hips, shoulder) and I therefore sleep better when I'm having the infusions.

Your husband may be able to do a couple of other things to avoid infections. My ANC is usually a lot lower than 900 - often only half that. However, since diagnosis with MDS I have only had 1 bladder and 2 chest infections - better than before I was diagnosed. A couple of other things I do which I believe help: I avoid foods containing added sweetening (sugar, maple syrup, etc) except for honey and stevia. If I feel the slightest hint of a sore throat I take 5000 mg Echinacea (capsule) and continue this a couple of times a day until the soreness disappears. The idea is to knock it before it goes to your chest. I believe being dairy free also helps because sugar + dairy creates phlegm and I think that phlegm is probably a good breeding ground for germs. With the bladder, I take 15,000 cranberry capsules if there is the slightest hint of it and this works too.

Best wishes! Will be interested to follow your posts as your husband is also the only other person I've come across so far with hypogammaglobulinaemia + predominating white cell problems.

[Barbara K]

Hi, Cheryl--Thanks so much for these further thoughts and suggestions. The material on that link was interesting--though it left me in chicken-and-egg mode wondering what is causing what for my husband, or at least how it all fits together.

So while I am very sorry we are both here (!), I am grateful you are willing to help, and as we get in more info in coming weeks I'll let you know. In the meantime, I'm going to have to get through this work week before I go back and read your old posts, but that's high on my weekend to-do list. And I've made notes on your suggestions for diet-related strategies. Fortunately, he takes really good care of his health and already avoids sugar. We might need to have a talk about the dairy intake, though!

Take care. It sounds like you've done an amazing job managing your health concerns.

[Barbara K]

Hi, Cheryl--A quick note. I had time this past weekend to read back through your old posts. One thing that popped out was a discussion you had w/ Chirley about copper levels and neutropenia/MDS symptoms. Not only was my husband's ferritin quite low (I think it got down to 5 at one point), but his ceruloplasmin (something like that--related to copper) was something like one point below the cut-off line for normal for the lab that did the results. His doctor didn't think that was low enough to cause neutropenia, but we have wondered what to make of the low iron and relatively low copper measurements. She chalks his low iron up to diet (mostly vegetarian plus fish) and blood donation; we're dubious about that, as he never donated that regularly, and unless he is taking massive doses of iron supplements his ferritin continually plummets. Something just doesn't seem right.

I found an old scholarly article from 1985 that said that hypogammaglobulenemia seems to be connected to low iron, and it speculated that malabsorption was the problem (though it offered other possible causal relationships as well). Our thinking is that it just seems to be too much of a coincidence that he would have ended up, as an otherwise very healthy and well nourished middle-aged man, with both iron deficiency and the other symptoms I have described, and that they would be unrelated. His colonoscopy and endoscopy--done before the BMB--gave normal results, though, as did various other kinds of testing from the digestive type docs.

At any rate . . . . if I learn anything more I'll let you know. When I first started reading this site I remember puzzling over Chirley's posts regarding her low copper, but I didn't post anything myself because my husband's level was just barely low and we are sitting, relatively speaking, on easy street.

All best--BK

[Chirley]

Hi, there was another lady on this site who used to post about her husbands low copper.

Her user name was Cathybee. You might be able to read through her old posts and get some info.

[Cheryl C]

Hi Barbara - You probably noticed from my posts that I am also vegetarian except for occasional fish and am also dairy free. My ferritin, while not high, is within normal range (92 ug/L a week ago). My iron is normal too (26 umol/L) as are B12 and folate. (I don't supplement except for Vit. D3). I don't think your husband's ferritin can be connected to diet unless he is not ingesting enough foods which enhance iron absorption or is perhaps eating/drinking things that prevent iron absorption.

You may have already checked all this out. If not, here's a useful and easy to read website that might help:
http://www.nomeatathlete.com/iron-for-vegetarians/

I am having an IgG infusion tomorrow as my globulins have dropped back below normal again.

[Barbara K]

Hi, Chirley and Cheryl--Thanks for these further tips.

I'll look for Cathybee's old posts this weekend, Chirley. I don't recall seeing her name off the top of my head, but probably I have read some of her posts. It can take a while until you realize that a given person's comments might be relevant to your situation--esp. when you don't quite know what your situation is.

And, yes, Cheryl, I don't see his ferritin and iron issues as likely to result from inadequate intake or absorption problems over which he has any control (he doesn't use caffeine, etc.). It sounds like hypogammaglobulenemia isn't necessarily the answer, though, given your numbers.

Chirley--I know you have been through many rough patches and hope you are doing OK, and, Cheryl, I hope your infusion goes smoothly. Barring some unforeseen development, I guess we are sitting tight until next appointment in early April. If she recommends another BMB, he'll definitely do it.

Take care.