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AA Aplastic anemia

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  #1  
Old Sat Dec 28, 2013, 02:38 PM
curlygirl curlygirl is offline
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Two New PubMed Abstracts Regarding the Role of PNH in AA

PubMed, http://www.ncbi.nlm.nih.gov/pubmed, the National Institute of Health's (NIH) online database has a bunch of new abstracts for articles regarding Aplastic Anemia published in November and December. Two in particular showed that having a PNH clone in addition to AA made you more likely to respond to Immunology-Suppressive Therapy (ATG & Cyclosporine) than if you don't have PNH. Here are the links.:

http://www.ncbi.nlm.nih.gov/pubmed/24261566

"The response rate at 6 months was higher in PNH+ patients than that in PNH- patients, both after first- and second-line IST: 68% vs. 45%, P = 0·0164 and 53% vs. 13%, P = 0·0502 respectively. Moreover, 42% of PNH+ patients achieved complete remission compared with only 16% of PNH- patients (P = 0·0029)."

http://www.ncbi.nlm.nih.gov/pubmed/24236830

"Among 22 children receiving immunosuppressive therapy (IST) for AA, 73% (16/22) were PNHpos, of whom 94% achieved at least a partial response (PR) to IST; 11/16 (69%) achieved complete response (CR). Only 2/6 (33%) PNHneg patients achieved PR."

I don't think the articles get into the why yet, just that they were determining a positive correlation. You can't read the entire article without a subscription but sometimes if I find a particularly interesting article and it's in a journal that my doctors get (e.g. the American Journal of Hematology) they can tell me the outcome of the entire article.
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Old Sat Dec 28, 2013, 08:36 PM
marmab marmab is offline
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Pnh & Aa

Thanks for passing along this information. It has personal relevance for me as I am one of those AA patients who does not have a PNH clone, and I had no response to two rounds of immunosuppression (hATG/CsA). It will be interesting to find out more about the correlation of PNH and AA.
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Marmab, F65, SAA/hypo MDS dx 7/2011. Tried ATG/CsA, IvIG, Rituxan, prednisone, Promacta -- none of these helped. Transfusion dependent until MUD BMT 7/17/14. Prep. regimen of Campath, Fludarabine & Cytoxan. Doing great. 100% engraftment. No GVHD.
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  #3  
Old Sat Dec 28, 2013, 09:54 PM
Heather8773 Heather8773 is offline
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My husband and I were told this and were hopeful that he would do well but he had no response w ATG. O well everything happens as it should and I guess he just wasn't one of those ppl it worked for. We were also told ATG could raise his PNH clone and it did. About 10 pts. From 18 to 28. But I'm happy you shared this bc it is true for many.
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Heather, wife of Ronald 36; dx PNH 2012; Dx VSAA 2013; eculizumab(Solaris) hATG 2/20/13 cyclosporine 400 mg daily. 37 units RBC and 15 units of platelets. Post BMT -pentam,vorconizole,valtrex, valcyte, actigall, Pepcid , prograf, magnesium.
10/10 MUD 10/10/13
Now no PNH or AA. Mixed Chimerisim
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  #4  
Old Sat Dec 28, 2013, 11:34 PM
triumphe64 triumphe64 is offline
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Quote:
Originally Posted by Heather8773 View Post
My husband and I were told this and were hopeful that he would do well but he had no response w ATG. O well everything happens as it should and I guess he just wasn't one of those ppl it worked for. We were also told ATG could raise his PNH clone and it did. About 10 pts. From 18 to 28. But I'm happy you shared this bc it is true for many.
Heather,
Plan to attend the Hope Step and a Cure walk on March 22 in Houston. Details will be posted after the first of the year on the AAMDS web site and on Marrowforums. There will be many patients there.
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Dallas, Texas - Age 81 - Pure Red Cell Aplasia began March 2005 - Tried IVIG - Then cyclosporine and prednisone. Then Danazol, was added. Then only Danazol . HG reached 16.3 March 2015. Taken off all meds. Facebook PRCA group https://www.facebook.com/groups/PureRedCellAplasia/
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  #5  
Old Thu Jan 2, 2014, 12:51 AM
Heather8773 Heather8773 is offline
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Quote:
Originally Posted by triumphe64 View Post
Heather,
Plan to attend the Hope Step and a Cure walk on March 22 in Houston. Details will be posted after the first of the year on the AAMDS web site and on Marrowforums. There will be many patients there.
Thank you I will sign up!
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Heather, wife of Ronald 36; dx PNH 2012; Dx VSAA 2013; eculizumab(Solaris) hATG 2/20/13 cyclosporine 400 mg daily. 37 units RBC and 15 units of platelets. Post BMT -pentam,vorconizole,valtrex, valcyte, actigall, Pepcid , prograf, magnesium.
10/10 MUD 10/10/13
Now no PNH or AA. Mixed Chimerisim
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