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PNH Paroxysmal nocturnal hemoglobinuria

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  #1  
Old Mon Nov 7, 2016, 12:55 PM
Stephanfl Stephanfl is offline
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Location: Miami, FL
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PNH - but there must be more

Hi Everyone!

I am new to this forum, and appreciate the wealth of information on here.
While I live in Florida and am not a patient, I am on here for my father-in-law, who was diagnosed with PNH in February 2016, and he lives in Switzerland.

His PNH is being treated with bi-weekly Soliris dosages, and he also needs blood transfusion (red cells), every 2-4 weeks.

Many tests have been done on him, but the doctors don't know why his red blood cells won't recover, neither do his platelets. They are now thinking about the marrow transplant, but really don't know what else he could have, in addition to the confirmed PNH.

There are a few things I know about him, that could contribute to his illness.
For one, he has had two artificial front teeth since childhood (70's) and I heard before that dentists used to use a "glue" containing Benzene. He still has those two teeth today.

He also used to love to build airplane models, using glue and paint, most likely to have contained Benzene.

He has been tested for MDS (and maybe other diseases, I am unaware of), but so far, only PNH has been confirmed.

If anyone here has an idea of what else other than Soliris could help him, or should be searched for, I would greatly appreciate it.
A few additional interesting facts I want to share here, just in case someone might see a relationship to anything:

- He lost most of his hair at a fairly young age (in his 30's)
- He has had issues with his skin for most of his life (dry skin, eczema)
- He, and two of his Kids have Asthma, and had it for decades
- He has a history of heartburn/burping
- One of his daughters has the autoimmune disease "Hashimoto's"
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Old Tue Nov 8, 2016, 10:43 AM
Marlene Marlene is offline
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Have they mentioned Aplastic Anemia? Usually, they must first rule out blood cancers, MDS and genetic/inherited anemia's like Fanconi's/Diamon Black-fan anemia or telomere issues with a the TERT/TERC mutations before diagnosing Aplastic Anemia. Many with AA have some level of PNH clone.

How is he treating his heartburn? The new heartburn medicines, like proton pump inhibitors, will cause nutritional issues over time which will impact ones health at some point. B12, folate, Copper, iron, zinc are the key blood nutrients affected here.
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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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Old Wed Nov 9, 2016, 08:57 PM
Stephanfl Stephanfl is offline
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Join Date: Nov 2016
Location: Miami, FL
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Hi Marlene,

Thank you so much for your response.
They did mention Aplastic Anemia, but I am not actually sure as to the severity of it, or how it was diagnosed. I will ask him about that.

What I do know is he has had at least 2 spinal tabs and a series of blood tests done, and they have ruled out any cancer and MDS everytime they tested.

They have also searched for things (I am sorry, I am not that medically adverse with the correct terms) in his DNA multiple times, without any indication of anything out of the ordinary.

I know that he was told that Soliris seems to be working fine as far as the "destruction" on his blood cells goes, but his blood counts still do drop. I have a few of his lab results actually, but am not sure which of the numbers would be of most interest to you?
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Old Fri Nov 11, 2016, 09:13 AM
Marlene Marlene is offline
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Join Date: Oct 2006
Location: Springfield, VA
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Aplastic Anemia is a diagnosis of exclusion and once they rule out all the other possibilities, they can then diagnose the anemia as aplastic anemia. Sometimes they call it pancytopenia meaning that all three blood lines are affected, red cells, platelets and white cells.

There are three diagnostic criteria of aplastic anemia:

Severe (SAA) - The bone marrow cellularity is <25% AND at least two of the following are met; Neutrophils <0.5; Platelets <20; and Reticulocytes <20.

Very Severe (VSAA) - Same as above but the neutrophils are <0.2

Moderate (which is not usually treated) - The bone marrow cellularity is <25% but the peripheral blood counts do not meet the criteria for SAA

Reticulocytes are new or immature red blood cells being made.

Hope this helps
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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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