pnh question

[3blankets]

Hi, I have had aa for the last three years and had horse atg in November of 08. Recently I was tested for PNH but it seems that only the white cells are affected. Has anyone ever heard of this? All I have read tells me that it should affect the red cells. If anyone has any experience with this, please let me know. Thanks

[Ryan Jay]

From what I understand...

PNH affects both whites and reds.

However, your red cells are what get ripped to shreds by your immune system. This makes them impossible to test for how profound your PNH is.

The whites are used for testing purposes because they don't get torn asunder like the red cells, although they do suffer from that missing protein. So, they serve as a PNH measuring stick, but it's the red cells that create the clinical issues. The clotting risk is not yet entirely understood.

[Andrya D]

There are several webinars on aamds.org that can explain it.

[nan]

PNH can affect all 3 cells or only one any one of them, red.white or platelets.It doesn't have to be all 3 either one. My husband it affects his red cells.he has had PNH since 1990 or maybe even earlier.

[3blankets]

Thanks for the replies. I was just tested again, and my red cells look normal but my whites are showing the signs of pnh. It's weird.

[Susan]

Erin, if you are referring to a recent flow cytometry test for percent of PNH clone, it is the WBCS which indicate the clone percent. Since RBCs are lost to hemolysis in all PNH patients, even if hemolysis is very minor or invisible ( no red/dark discoloration seen in urine), the pathologist only interprets using the WBCs affected.
Make sure a hematologist with a good PNH background reads the result for you, not uncommon for the inexperienced to misinterpret the data. You also want to have the test done at a facility which does PNH flow cytometry fairly often.

Since all PNH patients have an element of Aplastic Anemia (AA), it is relatively common for PNH patients to have reduced wbcs and platelets in addition to reduced rbcs from hemolysis and possibly from AA. The reticulocyte count and % will tell you if your marrow is producing enough rbcs. So will a bone marrow biospy but unless your counts are pretty low you may not need one.

Dr Rosse, retied from Duke calls PNH marrows "lazy." PNH patients are less likiely to develop empty ( nealy complete lack of cell production) AA marrow but it happens once in a while.

[3blankets]

Thanks.

I did just have another bone marrow biopsy. I think my 15-20% of my cells are pnh cells. I am not sure what the next step will be. My counts are stable right now, wbc 2.3 hematocrit 28, platelets 45,000. At what point does the solaris become necessary? I am also worried about blood clots. How would i know if i had one??

[Susan]

Soliris is used to help prevent blood clots and give you more energy when low hemaglobin and fatigue are caused by PNH hemolysis (red cells breaking down). It will prevent kidney damage. Studies are showing the Soliris is so good most PNHers can now live a normal life span! It is best to have a PNH specialist advise you on whether or not you need or will benefit from Soliris. he/she can review your past flow cytometry, tell you if done correctly or repeat it if not.

Susan

[Susan]

An MRI of the veins in the abdomen and head can rule out history of or current clots. Can occur in legs, but often signs, redness, pain, swelling. Usually you would know if you have one but sometimes can be silent. Watch the AAMDS webinars!

I hope you go see a PNH expert, well worth it. Most hematologists, even great one know very little about PNH. You'll feel much better once you see one, learn so much more for sure about your situation:-) They can work with your local hematologist.

Susan