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  #1  
Old Mon Jan 27, 2014, 12:26 AM
bachgalupe bachgalupe is offline
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Recently Diagnosed with Pure Red Cell Aplasia

I am a 41 year old male. I recently moved across the country to Arizona from NY. I was in relatively good health until I was diagnosed with Type 2 Diabetes in April of 2013. I listened to everything my doctor told me and I was controlling my Diabetes and began to lose weight and control everything through diet. Forward to September of 2013, when my wife, daughter, and I moved to Arizona. Everything went well until I had to move my washer and dryer out of my garage to another house. I felt like I was "winded" as if I ran a mile sprinting. I knew this was not normal, because with the Diabetes diagnosis I was hitting the gym fairly regularly. I called my on call Dr. as I thought there was something seriously wrong. I feared a heart attack or something else very serious. I noticed at complete rest, I was relatively fine. The on call Dr. had me make an appointment for my primary Dr. in the morning and I drove to see him the next day. There he checked me out and did some routine blood work. His initial diagnosis was, "You could be that out of shape, Ken." Here's a prescription for an inhaler. I'll get back to you on your blood work. Through the evening I still could not do anything without losing my breath and my heart beating like crazy. The next morning I got ready for work and as I was walking out the door the Dr. called me. He told me to have someone drive me to the closest Hospital and tell me where you are going so I can alert them. He told me my hemoglobin level was a 5.1. My wife drove me to the Hospital and was immediately admitted. I was given two units of blood and sent up to a room and then they began several tests. I met a Hematologist while there and he blew my mind with all kinds of Dr. mumbo jumbo. After three days of test being poked countless times. He ordered a Bone Marrow Biopsy Aspiration and put me on Prednisone. He was thinking I had Hemochromatosis as my Iron levels were through the roof. after another 2 units of blood and not much else they could do for me, they discharged me under the care of this Hematologist. After the results came back I went to his office and he said the Bone Marrow Biopsy gave him a lot of answers to questions. Ruling out Cancer and other things, but he had to wait an extended amount of time to find out about Hemochromatosis. He said that I also could have Myelodysplastic Syndrome. Meanwhile, I was going two times a week for blood tests. My hemoglobin dropped again to a 6.3 and we scheduled another transfusion. The morning of my transfusion I passed out three times in my kitchen and living room and got to ride in an ambulance to get those 2 units of blood. After being discharged the second time, I was still getting tested 2 times a week and it seemed like every friday I was getting a 2 unit transfusion. Went to the Dr.'s office several times and he wanted to order a second Bone Marrow Biopsy. This is when I became wary. A second one? What are we looking for now? Now I decided to get a second opinion. Because it was now December and I had no answers. I reached out to the Mayo Clinic in Scottsdale, AZ. They reviewed everything and they decided I could be seen by them. Meanwhile, it took so long to get an appointment at Mayo I got the second Bone Marrow Biopsy. This is where he came back with MDS and wanted to treat me with the drug ATG. He couldn't administer this drug and referred me to another hematologist. I set an appointment for that doctor, but knew on just after Christmas I was going to The Mayo Clinic and I would let them handle my care from there. So I did get the appointment with Mayo and got all the clinic notes and results from the two BMB's. The doctors at Mayo immediately said they were glad I sought out the second opinion. But reviewing my information they didn't think my diagnosis was correct. So they did a ton of blood tests and they also wanted to do another BMB, because there were some key tests that were not done. I had the third BMB and the Dr.'s at Mayo came back with the diagnosis of Pure Red Cell Aplasia. I have recently begun my treatment with 150mg of Cyclosporine. My iron levels are still through the roof and I'm sure there are many details I have missed. But I am very scared and nervous, because from what I understand it is extremely rare in someone my age. I feel like Mayo has got me pointed in the right direction. Fingers crossed. Any feedback or advice would be appreciated.
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  #2  
Old Mon Jan 27, 2014, 09:29 AM
Marlene Marlene is offline
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I can't speak to the red cell aplasia but there are one two on the site who will probably see this who can. We all know how scary these bone marrow diseases are and it's critical to have a doctor who's familiar in treating them. It's good you have gone for a second opinion.

In the meantime, did you find out if you have hemochromatosis? High iron is linked to Syndrome X, a metabolic syndrome which includes diabetes as one of the conditions. High iron can and will impact how well your bone marrow produces blood. How high is your iron and have they discussed therapy to reduce it?

High iron will also create imbalances in copper and zinc so hopefully they checked those.

We found that the more iron John got rid of the better he felt and was able to heal. His iron overload was due to transfusion.

Regarding your red cell transfusions...a good rule of thumb is to set a red cell transfusion goal that is high enough that you're not passing out or having symptoms. John's HGB threshold was 8.3. At that level, they would transfuse two units which would bring his HGB up to around 10. If they are only giving you two units when your HGB is at 6, that is probably not high enough for you. When John was first diagnosed his HGB was at 5.5. They gave him 5 units of red cells to get him up to a decent level. From then on, he was transfused when ever his HGB got down to 8.

Something that pretty much every finds helpful is get copies of all your labs so you see the results for yourself and can track the trends. You would be surprised at how many time there are abnormal results that get missed or overlooked.

There key nutrients important to blood production. They are Vitamin D3, K2, C, B12, folate, B6, zinc, iron and copper. You should have been checked for all of those. A low-normal B12 is not OK and needs to be addressed.

Hope you find this useful.

Marlene
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Marlene, wife to John DX w/SAA April 2002, Stable partial remission; Treated with High Dose Cytoxan, Johns Hopkins, June 2002. Final phlebotomy 11/2016. As of January 2017, FE is 233, HGB 11.7, WBC 5.1/ANC 4.0, Plts 146K.
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  #3  
Old Mon Jan 27, 2014, 11:17 PM
bachgalupe bachgalupe is offline
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Hemochromatosis came back as negative. My threshold has been anything under 8.0. With my previous Dr. it would take a few days because of a type and cross and scheduling. So ultimately I would be much lower. Now at Mayo, I test, get the results in around 20 minutes and can have a transfusion immediately if I need to. I have never heard of Syndrome X. I will mention it to the Dr.'s. Thanks so much for the reply.
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  #4  
Old Tue Jan 28, 2014, 03:11 PM
Marlene Marlene is offline
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If you haven't already, do a search on this site for pure red cell aplasia for all the threads containing those words. You might find some of the discussions helpful.
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Marlene, wife to John DX w/SAA April 2002, Stable partial remission; Treated with High Dose Cytoxan, Johns Hopkins, June 2002. Final phlebotomy 11/2016. As of January 2017, FE is 233, HGB 11.7, WBC 5.1/ANC 4.0, Plts 146K.
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  #5  
Old Wed Jan 29, 2014, 11:03 PM
bachgalupe bachgalupe is offline
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Thanks Marlene!
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  #6  
Old Thu Jan 30, 2014, 10:05 PM
triumphe64 triumphe64 is offline
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Hi,

I've been away, so I have missed my daily checks of Marrowforums.

Why don't you start with the following thread. I know I covered a lot and hate to retype.
Then we can get specific.

http://forums.marrowforums.org/showthread.php?t=3897
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Dallas, Texas - Age 78 - 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/

Last edited by triumphe64 : Fri Jan 31, 2014 at 12:42 AM. Reason: small changes to wording.
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  #7  
Old Fri Jan 31, 2014, 03:43 AM
triumphe64 triumphe64 is offline
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I just read your post in detail.
I got winded turning over in bed. I didn't think anything about it, but it all makes sense now.
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Dallas, Texas - Age 78 - 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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  #8  
Old Fri Jan 31, 2014, 04:16 AM
triumphe64 triumphe64 is offline
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I just remembered that there is an event for AAMDS this Sunday in Phoenix. There won't be any PRCA people, but there will be some others familiar with aplastic anemia, the next closest thing, and some reps from AAMDS. That will be helpful. It's too late to get a shirt, but a good way to start learning.

https://friendraising.towercare.com/...dCode=J9H2MHKS

Ron Duncan
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Dallas, Texas - Age 78 - 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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  #9  
Old Tue Nov 25, 2014, 01:04 PM
gina66 gina66 is offline
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PRCA due to inconclusive bone marrow biopsy

PRCA has been my diagnosis for 3 yrs. now. This dx is usually assigned if the RBC is the only one affected. My opinion with this dx is that you may be treated with meds. or chemo that were used for different dx like MDS, RA, etc. Red blood cell aplasia I think could maybe a precursor for a more specific disease later in life or maybe due from the chemo a patients were subjected during the course of this dx thus it may turn into MDS. I would suggest to see if doctors can give you bone marrow biopsy once a year. I had 3 BMB. There was a 2 yr. gap from the last I had last Nov. 10 at UCLA. I would suggest to have a bone marrow biopsy at a very good clinic. My first 2 BMB was inconclusive. There was some 2-3 % monoclonal B cell abnormalities but not significant for any specific disease. I had prednisone, cyclosporine, Cytoxan, dacogen, campath, and retuxin. Having PRCA you get to try with trial and error with different meds/tx used for different bone marrow and auto immune diseases. The result of my BMB at UCLA is still inconclusive. The monoclonal B cells abnormality is not found anymore which was probably killed by Rituximab. There is a bone marrow fibrosis(scarring of tissue) found which may mean different things. I continue to have blood transfusion mostly every 3 weeks and it has been for 3 years now. Dr. Paquette arranged for further genetic mutation testing for diseases. Until then, my dx is still PRCA. Good thing with me is that my health feels normal until my HGB goes down to 6.5 or below. Best of health to you.
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PRCA, infected by Parvovirus. Took Prednisone 20-60 mg/day for 6 mos., Cyclosporine 100- 200mg/day for 6 mos., Exjade 1000-2500 mg/day current, Warfarin 3-4mg/day, Cyclophosfomide 50 mg/day for 4.5mos., Campath for 2.5 mos, Danazol 200-400 mg/day for 6 mos. Currently- Rituximab for 4 wks.
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  #10  
Old Tue Nov 25, 2014, 02:31 PM
triumphe64 triumphe64 is offline
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gina66,

It's good to see your post, and I know Dr. Paquette is excellent.

They found PRCA on my third BMB after they sent the sample to UT Southwestern. I think the prior pathologists were not smart enough to look for it. Now confirmed at UCLA and Cleveland Clinic.

Dr. Maciejewski is also looking into genetic testing for all his patients. He is also looking into every virus in the world as possible causes for bone marrow failures. This study will take time.

BTW; My blood has been running into 15.0 etc. I am only taking Danazol, and am down to only 50mg per day.
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Dallas, Texas - Age 78 - 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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  #11  
Old Tue Dec 2, 2014, 09:29 PM
gina66 gina66 is offline
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genetic mutation testing at UCLA

Triumphe64,

Nice to hear your HGB has been normal for a while now. BTW, Dr. Paquette told me that this testing that he ordered for me is pretty new. It is important to have the right dx to get the right tx. I will let you know about the result.
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PRCA, infected by Parvovirus. Took Prednisone 20-60 mg/day for 6 mos., Cyclosporine 100- 200mg/day for 6 mos., Exjade 1000-2500 mg/day current, Warfarin 3-4mg/day, Cyclophosfomide 50 mg/day for 4.5mos., Campath for 2.5 mos, Danazol 200-400 mg/day for 6 mos. Currently- Rituximab for 4 wks.
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  #12  
Old Mon Feb 9, 2015, 08:00 PM
gina66 gina66 is offline
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Triumphe66,

I had ATG at UCLA last Jan. 5 - Jan 9. I did not get serum sickness. Cyclosporine regimen for 300 mg a day for now. It is going to take months to see results. Dr. M planned this for me also but because I have family in California, I decided to have it UCLA and other appointments in the future. Did you have ATG tx also?
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PRCA, infected by Parvovirus. Took Prednisone 20-60 mg/day for 6 mos., Cyclosporine 100- 200mg/day for 6 mos., Exjade 1000-2500 mg/day current, Warfarin 3-4mg/day, Cyclophosfomide 50 mg/day for 4.5mos., Campath for 2.5 mos, Danazol 200-400 mg/day for 6 mos. Currently- Rituximab for 4 wks.
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  #13  
Old Mon Feb 9, 2015, 08:02 PM
gina66 gina66 is offline
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The result of the genetic mutation testing and T cell testing did not show any abnormalities. PRCA dx is still my dx.
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PRCA, infected by Parvovirus. Took Prednisone 20-60 mg/day for 6 mos., Cyclosporine 100- 200mg/day for 6 mos., Exjade 1000-2500 mg/day current, Warfarin 3-4mg/day, Cyclophosfomide 50 mg/day for 4.5mos., Campath for 2.5 mos, Danazol 200-400 mg/day for 6 mos. Currently- Rituximab for 4 wks.
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  #14  
Old Mon Feb 9, 2015, 11:48 PM
triumphe64 triumphe64 is offline
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Quote:
Originally Posted by gina66 View Post
Triumphe66,

I had ATG at UCLA last Jan. 5 - Jan 9. I did not get serum sickness. Cyclosporine regimen for 300 mg a day for now. It is going to take months to see results. Dr. M planned this for me also but because I have family in California, I decided to have it UCLA and other appointments in the future. Did you have ATG tx also?
I did not have ATG. Good luck. It will be worth the wait.
__________________
Dallas, Texas - Age 78 - 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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  #15  
Old Sat Aug 13, 2016, 01:05 AM
gina66 gina66 is offline
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Red blood cell Aplasia

Since my last post, I have taken Methotrexate (med for RA). That did not work, I took it for 7 mos. Then Dr. V from MD Anderson asked me to stopped taking it. 4/17/16, I tried this supplement called "redox signaling molecules" by ASEA. Dr. V then prescribed Sirolimus for 1.5 mg. I think the supplement help my hemoglobin going down slowly and with the Sirolimus, the two works wonder. Since July 2016 my hemoglobin went up to 12.7 until now it's normal even though the Sirolimus is reduced to 1 mg. Anybody who is interested can research on my site www.cellhelpbyASEA.teamasea.com. Who knows it may help you also. There is a research on this site. You can research also for scholarly research for "redox signaling molecules". I hope it would help you also

08/09/2016 Retic Count, Blood Normal Reticulocyte 0.7 % 0.5-2.0 % Final
08/09/2016 Cbc Normal Wbc 5.5 TH/mm3 4.9-10.1 TH/mm3 Final
Normal Rbc 4.14 mil/mm3 4.10-5.50 mil/mm3 Final
Normal Hgb 13.0 g/dL 11.9-16.1 g/dL Final
Normal Hct 38.3 % 36.9-47.1 % Final
Normal Mcv 92.5 fL 79.1-100.0 fL Final
Normal Mch 31.3 pg 26.9-32.1 pg Final
Normal Mchc 33.8 g/dL 31.9-36.1 g/dL Final
High Rdw 14.8 % 11.5-14.5 % Final
Normal Platelet 315 TH/mm3 150-450 TH/mm3 Final
Low Mpv 6.1 fL 7.5-11.0 fL Final
Normal Absolute Neutrophil Count 3.70 TH/mm3 2.00-7.00 TH/mm3 Final
08/09/2016 Wbc Diff, Auto, Blood Normal Gran 67.9 % 35.0-85.0 % Final
Normal Lymph 21.8 % 20.0-55.0 % Final
Normal Mono 5.3 % 2.0-10.0 % Final
Normal Eos 3.9 % 0.0-11.0 % Final
Normal Baso 1.1 % 0.0-2.0 % Final
__________________
PRCA, infected by Parvovirus. Took Prednisone 20-60 mg/day for 6 mos., Cyclosporine 100- 200mg/day for 6 mos., Exjade 1000-2500 mg/day current, Warfarin 3-4mg/day, Cyclophosfomide 50 mg/day for 4.5mos., Campath for 2.5 mos, Danazol 200-400 mg/day for 6 mos. Currently- Rituximab for 4 wks.
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