I have not done this in years....but a Physician is asking us to perform testing to see if a patient has PCH. What I have done historically, is the Donath-Landsteiner Test.....but neither ARC or Mayo Clinic seem to even do it as a send-out test.

1.  Does anyone else still test for PCH?

2.  Is there a "new" Test Protocol out there for PCH that I am just not familiar with?

 

Thanks,  

Brenda Hutson

We've never had an order for it, but I know my local reference lab offers it as test if we needed. As far as I know that's still the definitive test.

We still do it Brenda.

 

The only thing that we do slightly differently these days is to do the indirect two-stage Donath-Landsteiner (using fresh complement from a person who is ABO compatible and with no known atypical antibodies), just in case the patient's own complement has been consumed in vivo.

The Technical Manual has instructions.  

Right...and it looks like I will end up just doing it myself.

Brenda

 

The Technical Manual has instructions.  

Judd also has the procedure in his latest book.

Don't worry Brenda

It's really not difficult - and it gives you the feeling you've gone back in time and are doing 'real' lab work rather than pushing a button.  And SOOOOO nice when you get a positive (well, not for the patient, though).

Well, negative Donath-Landsteiner....but problematic patient nevertheless.

History at another Facility of Warm Auto in Eluate only.  Yet patient had hemolytic transfusion reactions.  So when we got her, we asked for the other Institution to Fax their work-up.  They had also done a complete phenotype on her (yeah).  We obtained a panagglutinin in the Eluate; and probably 2/3 of the cells in the plasma work-up were reactive.  We don't do adsorptions here (minimal staffing and multiple Generalists).  I figured that between her negative reactions and the phenotype, we could at least obtain some blood for her.  We could not rule-out a possible G; with or without C (patient is Rh negative).  Even though we had some homozygous Jka cells that were non-reactive, we also had some that were reactive (and I have seen too many of them that are "hit and miss;" even on homozygous cells, when using GEL).  So given her history, I had decided to give Jka Negative anyway.  But that only left us an E, K and Fyb to worry about.....so I decided to just give her phenotypically matched blood.  But guess what.....she had a hemolytic reaction anyway!  The physician, knowing her history, actually stopped the transfusion after 150 cc's and assessed her urine; and there was a significant amount of blood (plus her Hct dropped after the transfusion).

So, my conclusion (and I think the Physician's at this point) is that her Warm Auto is causing the hemolysis (and it may very well be that what we are seeing in the plasma is just the Warm Auto spilling over; but would have had to do adsorption studies to tell for certain). So the Physician is going to try to just give her steroids for awhile and stop transfusing.

Brenda

Hmmmmmmmmmmmm. I would be surprised if a warm-reacting auto would cause an immediate haemolytic transfusion reaction like that Brenda.

I still think that there is something else going on, but quite what I don't know.

I am just wondering about a possible anti-Vel (I know, I know, it is VERY unlikely). They tend to be IgM, so are not detected brilliantly by gel techniques, but are very clinically significant. The other thought is a possible antibody within the Dombrock Blood Group System?????????????????????

 Has she had several reactions, or just once?  Could it be that the blood was infected?  And - 'patient had haemolytic transfusion reactions' - on what basis was that decision made? Has anyone actually proved that she is haemolysing?  And if so, that that was directly realted to a transfusion?  Has she received IVIg? Could this be due to something unrelated to IH?  Favism maybe?

 Has she had several reactions, or just once?  Could it be that the blood was infected?  And - 'patient had haemolytic transfusion reactions' - on what basis was that decision made? Has anyone actually proved that she is haemolysing?  And if so, that that was directly realted to a transfusion?  Has she received IVIg? Could this be due to something unrelated to IH?  Favism maybe?

 

Patient hemolyzes every time she is transfused (all lab data supporting it; plus they stopped her transfusion we gave her, after 150 cc's and obtained a urine sample and it was red).

Brenda

Then that sounds more like hyperhaemolysis syndrome - very common in Sicklers but not unknown in others

Anna

Then that sounds more like hyperhaemolysis syndrome - very common in Sicklers but not unknown in others

Anna

I don't know that her hemoglobin ended up "lower" than it was initially....but she certainly never increases.

If she comes back to us, I will probably send her to reference lab for a High Incidence work-up (just to be on safe side). 

This is one of those cases in which I would have called Dr. Garratty....and he would have graciously assisted me.

Brenda

It could be hyperhaemolysis, but I have my doubts.  The reason I say this is because patients with hyperhaemolysis either tend to have reactions that get worse and worse (and I include fatal reactions in this), or, for some unknown reason, tend to tolerate subsequent transfusions without turning a hair; but that does not mean that there are not patients that go down the "middle road" - it's just that they are very rare.

 

I would fall down on the side of an antibody directed against a high prevalence antigen.  However, I think I suggested a possible anti-Vel earlier in this thread.  I am now thinking more in terms of an e-variant who has made an "alloanti-e" or, worse, something like a Partial D Category 3, who has made an alloanti-D.

 

I realise that I have recommended molecular techniques a bit liberally on this site (probably because I don't have to pay for them!!!!!!! - although I do have to justify them), but I really think that full examination of the RHD and RHCE genes at a molecular level may help in this case (although it could equally be an antibody directed against another high prevalence antigen, such as anti-Jsb or anti-Fy3).  Incidentally, contrary to what many people think, homozygosity for the GATA-1 mutation and a Duffy genotype of FY*B/FY*B or FY*/FY*B does not completely exclude the possibility of forming an anlloanti-Fy3.

 

Have the clinicians tried transfusion covered by IVIG and high-dose methylprednisolone?  That may help in the short term?

I think molecular testing is warranted in this case. 

It could be hyperhaemolysis, but I have my doubts.  The reason I say this is because patients with hyperhaemolysis either tend to have reactions that get worse and worse (and I include fatal reactions in this), or, for some unknown reason, tend to tolerate subsequent transfusions without turning a hair; but that does not mean that there are not patients that go down the "middle road" - it's just that they are very rare.

 

I would fall down on the side of an antibody directed against a high prevalence antigen.  However, I think I suggested a possible anti-Vel earlier in this thread.  I am now thinking more in terms of an e-variant who has made an "alloanti-e" or, worse, something like a Partial D Category 3, who has made an alloanti-D.

 

I realise that I have recommended molecular techniques a bit liberally on this site (probably because I don't have to pay for them!!!!!!! - although I do have to justify them), but I really think that full examination of the RHD and RHCE genes at a molecular level may help in this case (although it could equally be an antibody directed against another high prevalence antigen, such as anti-Jsb or anti-Fy3).  Incidentally, contrary to what many people think, homozygosity for the GATA-1 mutation and a Duffy genotype of FY*B/FY*B or FY*/FY*B does not completely exclude the possibility of forming an anlloanti-Fy3.

 

Have the clinicians tried transfusion covered by IVIG and high-dose methylprednisolone?  That may help in the short term?

 

It may be thought the patient is a sickle cell patient because someone suggested possible "hyperhemolysis;" but she is not; and she is caucasian (and my experience with hyperhemolysis has been in this patient population). 

Also, she is Rh NEG.

Though the previous Institution obtained a panagglutitin in the Eluate only (and called it out as a Warm Auto), we also obtained Plasma reactions (probably about 4/5 of the cells).  It could have been the Warm Auto spilling over....but based on the patient's phenotype (which the previous Institution had thankfully already performed), we could not rule-out Anti-C and or G; and possibly a Jka hitting only some of the homozygous cells (GEL).  But we elected to give Pheno-matched anyway (D-C-E-, K-, Jka-, Fyb-).  With all cells in the Eluate reacting, the though of a High Incidence did occur to me (and could still be a possibility); but the Plasma did not appear to be the same (though we only performed GEL testing). 

I had really hoped she would do ok with the pheno-matched....so if she comes back to us, I may have to send her to the National ARC Reference Lab for further work.

And yes, she is now on steroids (not sure if prednisone or something else).

Thanks,

Keep those Thinking Caps on!  

Brenda