PNH - blood test

[rcusher]

I just received this PNH test result and was wondering if anyone knows what it was testing for. I don't see my doctor until next week and I am curious.

Thanks,

Jack

INTERPRETATION Flow Cytometry analysis did not detect RBC and WBC with PNH-associated phenotype. RBC, Granulocytes and Monocytes show normal expression of the GPI-linked molecules and GPI-linker. . FLOW CYTOMETRIC ANALYSIS RESULTS: GPI-DEFICIENT WBCs (FLAER-/CD157-): CD15+ neutrophils: < 0.1 % CD64+ monocytes: < 0.1 % GPI-DEFICIENT RBCs (CD235a+/CD59-) Type III RBCs: < 0.01 % Type II RBCs : < 0.01 % Total GPI-deficient RBCs: < 0.01%

[cajunjeff]

Rcusher, I think pnh is an acronym for paroxysmal nocturnal hemoglobinuria, a rare blood disorder that can result in hemoglobin in your urine that makes your pee look dark. Pnh can cause AIHA and kidney disease.

Your flow cytometry test appears to confirm you dont have pnh, which is good.

Why did your doctor ask for this test? I don’t know. Maybe you had a urinalysis that had excess hemoglobin, maybe you reported issues with urine color or maybe your hemoglobin is low and your doc was investigating. I would purely be guessing as to why this test was ordered.

I think good questions for your doc when you see him/her would be why was the test run and what were the results. I am assuming your test was negative for pnh because of the report language (but I am certainly no expert in interpreting this test result language):

INTERPRETATION Flow Cytometry analysis did not detect RBC and WBC with PNH-associated phenotype.

I hope when you find out you will update this post as to why the test was run. Good luck, here is an article discussing pnh:

my.clevelandclinic.org/heal...

[rcusher in reply to cajunjeff]

I am on the Loxo-305 clinical trial but was randomized to the Idelalisib + Rituximab arm. But after 6 months I developed drug induced colitis plus some other complications which landed me in the hospital for 10 days and had to stop the Idelalisib last October. It has taken me until now to be transferred over to Pirtobrutinib next week. So I am guessing this along with numerous other test is a part of the trial.

Will keep updated.

[cajunjeff in reply to rcusher]

That’s interesting. I wouldn’t think of pnh as being associated with cll to the point that a clinical trial would test for it, just because I haven’t heard of it much at all. I did some more reading and learned pnh is actually an acquired, rare type of hemolytic anemia.

I have had hemolytic anemia but I guess I did not have the pnh version. I do wonder if your doc did the test as part from the routine protocol of the clinical trial, or if there was something about your hemoglobin or haptoglobin levels that prompted the test. Or maybe pnh is some known complication of one of the meds in the trial.

I think of all the inhibitor drugs we can take like venetoclax (bcl-2 drug), ibrutinib (btk class drug) or idelalisib ( p13k class drug), idelalisib is the one with the most and more serious effects. Idelalisib is known to cause colitis and its not as well tolerated as other inhibitors, although all the them have side effects.

There is a big search for drugs we can take , short of transplants and carT therapy, for those of us who fail bcl and btk drugs, or are intolerant to them.

It seems like our best options now are idelalisib and non covalent btk drugs like pirtobrutinib. I am supposing in this trial, the makers of pirto want to establish its a better option than idelalisib, and that is what I guess the trial will show.

Due to its side effect profile, idelalisib is not used that much with cll compared to other inhibitors, and usually only after other inhibitors stop working. I am sure they are working hard to develop second generation p13k drugs with less side effects so we can have another option.

It’s good you got switched to pirto side of the trial. Most everything I read on it is positive, it’s just a question of how much time it buys us and what drug will be up next after it.

[AussieNeil Administrator in reply to cajunjeff]

This post from a year ago, summarises the situation with CLL treatments/trials with next generation PI3k inhibitor drugs: healthunlocked.com/cllsuppo...

In summary, in the USA, the FDA has been withdrawing the approval of previously approved indications for the use of these drugs for the treatment of CLL and is very closely examining the results from clinical trials for second generation versions. CLL specialists are continuing to research how they might be used more safely.

Neil

[rcusher in reply to cajunjeff]

I just had my appointment with my doctor and asked her why she had ordered a PNH blood test. Well she saw some abnormalities with my RBC's and also my haptoglobin test was also abnormal. It was done out the abundance of caution just to rule PNH out. After all that it was ruled out and all is okay. I start Pirtobrutinib tomorrow.🤞

[cajunjeff in reply to rcusher]

I’ was hoping you would update, thanks, all good news. Thats what I figured. I couldn’t see how a pnh test would be routine part of a clinical trial. I thought it might have to do with your hemoglobin or haptoglobin levels.

If you will permit me a longer response, I can tell you how I even know what haptoglobin is. I was about two yrs out from my Cll diagnosis when my local dr suggested to I might have hemolytic anemia (AIHA). WTH? I only knew AIHA as a complication of severe cll, I had good markers, so that cant be me.

So I had a trip planned to see my son in Argentina around that time and had an appt with a Cll doctor in Maryland at the NIH to join a clinical trial. They did all sorts of test there.

After my test I met with a dcotor at NIH there who I only later learned is quite famous in the cll world. I asked him if he thought I had AIHA. He said maybe I do, but my haptoglobin looked okay, so probably not. Then he told me what haptoglobin is and how it relates to AIHA.

While it seemed he thought I might not have AIHA, when I asked him if it was safe for me to go to Argentina the next month, his struggle to give me a firm yes or no answer made me think he just was not sure if I had AIHA or not.

End of story: I went to Argentina and had a blast. Shortly after I returned home, however, my hemoglobin crashed and I was profoundly ill. I was fortunate that didnt happen in Argentina.

Sorry to ramble. But your post had me research pnh which led me to AIHA which led me to this trip down AIHA memory lane.

I’m so glad you are negative for pnh. And I’m glad you got switched to what arm of the trial I would rather have been on. I hope you will post your experience with pirto and that it works for you for a very long time.

[roxieixor]

This means you do not have Paroxysmal Nocturnal Hemoglobinuria. PNH is an acquired genetic mutation where blood cells are deficient in glycosyl phosphatidylinositol (GPI)-anchored proteins which results in hemolysis as the red blood cells are destroyed by the complement system

Your flow cytometry report shows that your blood cells are not deficient in the GPI anchored proteins. Your PNH clone size is less than .1% which means you do not have PNH. Typically people with PNH clone size above 50% would need treatment to prevent the risk of blood clots from hemolysis.

If your RBC and Hemoglobin are low that could be a reason for ordering this test, but usually after eliminating other causes of anemia.

[rcusher]

Thanks for your reply, yes my RBC and hemoglobin are low. I have had numerous other tests and probably checking for anemia because of all the troubles I have had coming of the Idelalisib.

I can't say enough about my trial team of doctors at Princess Margaret hospital here in Toronto they are not leaving any stone unturned and I feel very comfortable with my treatment so far.

Jack