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AA Aplastic anemia

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  #1  
Old Sat Aug 24, 2013, 10:41 PM
curlygirl curlygirl is offline
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Red blood cell Morphology

Hello! As I mentioned in other threads, my son was diagnosed with Aplastic Anemia in April 2013, and had ATG in July 2013. So he is one month post-ATG and on cyclosporine (Neoral). I've been reading a lot about Aplastic Anemia, bone marrow failure, and auo-immune diseases in general, but for some reason tonight was the fist night that I landed on the National Hematologic Diseases Information Service (NHDIS) website: http://hematologic.niddk.nih.gov/aplasticanemia.aspx. One thing that caught my eye was the sentence: "Although production of mature blood cells is seriously impaired in aplastic anemia, the few blood cells that mature and enter the bloodstream are normal."

My son's Red Blood Cell (RBC) morphology has been listed as "normal" on his manual differential at exactly two visits in five months: one visit one week after ATG, when he was on Prednisone, and one visit last week while he was on Acyclovir. (He developed a nasty virus and had to be hospitalized.) Also, while on both of those medicines his WBC count increased. They decreased back to pre-medicine levels after going off the Prednisone and Acyclovir. At every other visits he's had "abnormal" red blood cells, including: Macrocytes, Microcytes, Ovalocytes, Schistocytes, Tear Drop Cells, and Hypochromasia, often including all of them at the same visit. I just wanted to make sure that other people are seeing these. Our hematologists said that they have seen this before, and I figured that it was a function of my son's bone marrow just trying to throw out whatever it can since it is under stress. I also thought it may have been because the packed RBCs used for transfusions are frozen and may undergo some abnormalities as they are thawed. So the article threw me off when it said that the RBCs should be "normal."

Thanks!
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Old Sun Aug 25, 2013, 02:02 PM
Hopeful Hopeful is offline
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Hi curlygirl,

I've heard from Dr. Young that mild red cell dysplasia can be seen with Aplastic Anemia. There are many articles that will mention this as well. So I wouldn't be concerned about abnormal RBC shapes in the blood, especially if he is transfusion dependent. If the WBC's or platelets also look abnormal, that may be more of a concern.
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52 yo female, dx 9/08, AA/hypo-MDS, subclinical PNH, ATG/CsA 12/08, partial response. Tried slow cyclosporine taper over 4+ years. Platelets fell, so back on cyclosporine. Trisomy 6 clone in 5% of cells.
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  #3  
Old Sun Aug 25, 2013, 02:11 PM
KMac KMac is offline
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Hi Curlygirl,

Those are excellent questions, and ones I've had myself.

In short, I believe the answers are 'yes' to all of them. When I was on prednisone, it raised my WBC, and the same thing when I get sick. My doctor feels that is a good sign. Even though my WBC runs quite low usually, it rises when I get sick. It shows the marrow has a reserve of white cells when needed. For example, last month I had a sore throat and my ANC was 2040. It clear up on its own without treatment. This month I feel better, and my ANC was only 560 last week.

And with 'abnormal' cells, I think when they say the cells are normal with SAA, they mean genetically normal (i.e. no cells that signify MDS, PNH, or leukemia). But morphologically, they are often still of unusual shapes and sizes, as the marrow fights to produce whatever it can. I go into more detail specifically about my experience with that on my Aug 21st posts in the 'Headache after ATG, on oral steroids and cyclosporine' thread, if you want take a look at that.

In the months immediately following ATG, my CBC reports almost always showed abnormal cells. Now, 18 months post-treatment, the morphology of my blood is much more normal.

Also, I did have abnormal platelets too, in that they were often small or fragmented. At least one other SAA patient at my hospital had abnormal platelets, and our doctor did not feel it was a cause for additional concern.
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Kevin, male age 45; dx SAA 02/2012 - Hgb 5.8, platelets 14, ANC 200, 1% cellularity. Received ATG 03/2012. As of 03/2015, significant improvement - Hgb 15, platelets 158, ANC fluctuates around 1000, Lymphocytes 620. Tapering cyclosporine. BMB 20-30% cellularity.
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  #4  
Old Sun Aug 25, 2013, 02:42 PM
curlygirl curlygirl is offline
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Kevin & Hopeful,
Thank you both! Your answers make me feel much better! Hopefully my son will stay healthy and he will go into remission soon. My son had 0.8% nucleated red blood cells (NRBCs) and a metamylocyte while on the predinsone, but when he went of the prednisone they went away. Last week on the acyclovir while he was admitted, he had 2 NRBCs for two days, and his reticulocyte count went up above 1. I'm waiting to see if this hangs around or goes away at tomorrow's visit now that he's off the acyclovir. It would have been nice for him to stay on the acyclovir if for no other reason than it was clearing up the two little plantar warts he's had on his foot for months and now they are getting bigger again.
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  #5  
Old Sun Aug 25, 2013, 11:09 PM
evansmom evansmom is offline
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FYI, cyclosporin can damage the lining of the microscopic blood vessels causing platelets and fibrin to be sequestered to those areas. As red blood cells travel, the fibrin threads that criss-cross the vessels cut the red cells in half. These mechanically damaged red blood cells are called schistocytes. The damage can also result in misshapen red cells. Premature demise of red blood cells can result in the release of immature red blood cells from the marrow. If the hemoglobin is not dropping at an unreasonably fast rate and the LDH is normal, this damage is tolerable. If transfusions are required more frequently than is expected and the LDH is elevated, this could indicate the cyclosporine's side effects are becoming dangerous.

One can Google "cyclosporine (or tacrolimus) associated microangiopathy".
This side effect post BMT was my son's only complication and it could have been deadly as he experienced severe microangiopathy and at it's worst, needed red cell transfusions every few days.
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Nicole, mom to Evan (20); diagnosed SAA November 2007, hATG mid-November 2007, no response after 6 months, unrelated 9/10 BMT June 2008, no GVH, health completely restored thanks to our beloved donor Bryan from Tennessee.

www.caringbridge.org/visit/evanmacneil
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  #6  
Old Mon Aug 26, 2013, 09:08 PM
curlygirl curlygirl is offline
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WBC went down :-( but reticulocytes are up to 2.45% :-) Hopefully they will stick around! I read that they can go up and down, too. Still had over over 25% each of macrocytes, microcytes, and schistocytes.

Evansmom, my son had massive hemolysis during his virus. One day he went from Hgb 8.4 to 6.4 overnight. He was getting PRBC transfusions every 5 days during those 2 weeks instead of every week and a half (his usual schedule.) And his bilirubin shot up to 1.7, confirming the hemolysis. Luckily that went away with the virus, too: he made it a week and a half until today, when his Hgb was 6.5 and his bilirubin was 0.6. Thank you for the feedback so that I know what to look for! I'm so glad I found this site and you're all so wonderful! My son asked today what would happen if his treatment didn't work and I told him not to worry, there were other options, like doing this again or having a bone marrow transplant. If someone had told me 5 months ago that having these options would make me feel better I wouldn't have believed it!
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  #7  
Old Thu Aug 29, 2013, 09:09 PM
curlygirl curlygirl is offline
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Evansmom,

The doctors tested my son's LDH today (without me mentioning it, I forgot) and it was over 800! We also did a first morning urine test that came up negative for blood in the urine. They were testing these to check on hemolysis as a result of the PNH clone that he has. They plan to keep him on the Cyclosporine level he is on.

Reticulocytes went down to 1.65% which is slightly disappointing but still much better that prior to ATG.

Thanks again!
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  #8  
Old Fri Aug 30, 2013, 09:12 PM
evansmom evansmom is offline
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Sounds like your son's virus provoked a hemolytic crisis secondary to his PNH. What is his clone size? In addition, it does sound like the cyclosporine is causing a bit of microvascular damage because, if I understand it correctly, that is why he has some schistocytes in his smear. Be aware that PNH and microangiopathy together increase the risk of blood clots. Not to add to your worry but it's something to keep in mind.

It's so sad our kids have to ask questions like "what if my treatment doesn't work?". All that worry on such little shoulders but you're right, there are options that will restore his health completely. Stay positive!
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Nicole, mom to Evan (20); diagnosed SAA November 2007, hATG mid-November 2007, no response after 6 months, unrelated 9/10 BMT June 2008, no GVH, health completely restored thanks to our beloved donor Bryan from Tennessee.

www.caringbridge.org/visit/evanmacneil
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  #9  
Old Sun Sep 1, 2013, 01:12 PM
curlygirl curlygirl is offline
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Hi, Evansmom, he had a 19% PNH clone prior to ATG. We haven't checked it again yet. Hopefully it will get smaller, not larger!
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