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Patients with Sickle Cell Disease


rravkin@aol.com

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Hi Everyone,

I have been wanting to post this for a while. I have worked in two different facilities which provide blood products for the patient with Sickel Cell Disease. However, one facility did a full phenotyping of the patent upon entry into the services they provided and the other facility did not. The benefit for the patient where the full phenotype was done is that they would then be given phenotypically matched blood and avoid the generation of antibodies which may complicate any urgent blood needs. Tell me about your practice.

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We would perform or refer the patient sample for full phenotyping but would only select blood for crossmatching based on the Rh and K typing (and obviously dependent on any other antibodies present). Since many of these patients develop multiple alloantibodies if they are subsequently transfused, then having the patient phenotype on file helps to determine these.

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We prefer our hospitals to send in samples for full phenotyping, even though we only recommend Rh and K matched blood, unless another antibody is present.

Knowing the full type helps us immensely. For example, on Wednesday, I had a lovely little problem case (HbSS with sepsis, Hb 6.7g/dL), during the night, of course, that had anti-S+Fya+Jka, + an anti-Fy3 reactinig with papain-treated red cells only. Without the full phenotype, I would probably still be working on it now (Monday).

:):):):):)

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I too work at two different facilities, one a children's hospital, where we do a full phenotype on all are sickle patients. We give Rh and K matched units with the exception of little e which we only give if the antibody is present.

If the patient presents to us without being a candidate to phenotype(transfused in the last 3 months) we will give C, E and K neg units until we can get a isotonic saline wash phenotype completed. Also we follow this protocal on an emergent basis if there is not a phenotype available. This way we do not have to delay transfusion waiting on the phenotype. We do always try to do the Rh's and K prior to transfusion is time permits.

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I have some experience in this in that I was the Supervisor of a Reference Lab in an area with a lot of sickle cell patients. In addition, I also gave a talk at a Seminar on the Laboratory aspect of treating sickle cell patients (followed a physician of Children's Hospital who presented the clinical aspects).

Anyway, the standard of practice is to do a complete phentoype when you initially see the patient (may need to do a hypotonic wash if they have been transfused in past 3 months; but if that is the case, I would first ask where they were transfused as there is a good chance that they did a complete phenotype and you can get the information from them). You then give them Rh and K system phenotypically matched (which "usually" means C-E-K-; but don't assume that as did a Physician in my current position when he first saw a sickle cell patient here). When and if they produce any alloantibodies, you then give them complete phenotypically matched RBCs (major blood groups). And of course, always Hemoglobin S NEG RBCs.

Hope that helps,:)

Brenda Hutson, CLS(ASCP)SBB

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We hardly ever get to do this on sickle cell patients, because our hematologists do not seem to understand the concept of the above procedures. They send them into the ED in the middle of the night, order 2 stat units, nobody informs the BB that they are sickle cell patients, we find out days later, and now they've been transfused. Grrrrr....

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...give them Rh and K system phenotypically matched (which "usually" means C-E-K-; but don't assume that as did a Physician in my current position when he first saw a sickle cell patient here).

Thanks Brenda for pointing out that not all sickle patients are C-E-. At our ref lab we see several R1R1 and a couple R2R2 with the correcsponding allos because the hospitals gave them CEK neg units with no regard to their actual phenotype.

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We hardly ever get to do this on sickle cell patients, because our hematologists do not seem to understand the concept of the above procedures. They send them into the ED in the middle of the night, order 2 stat units, nobody informs the BB that they are sickle cell patients, we find out days later, and now they've been transfused. Grrrrr....

You are correct; that is a problem in some Hospitals which rarely treat these patientsl; the Physicians are not aware of those requirements. I guess one thing that helps us is that the Order that prints out from our interface, lists the diagnosis (unless of course you work somewhere like I did once where the diagnosis was really the presenting symptoms; i.e. patient nauseous; so what if the reason they are nauseous is because they are undergoing chemotherapy for Leukemia and are a possible Bone Marrow Transplant patient who should have special requirements).!

At my current position, I have had to call Physicians a couple of times when we received orders on patients with a diagnosis that should have required special blood products. Basically, it was me telling them what to order based on the standard of practice.

Brenda Hutson, CLS(ASCP)SBB

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You are correct; that is a problem in some Hospitals which rarely treat these patientsl; the Physicians are not aware of those requirements. I guess one thing that helps us is that the Order that prints out from our interface, lists the diagnosis (unless of course you work somewhere like I did once where the diagnosis was really the presenting symptoms; i.e. patient nauseous; so what if the reason they are nauseous is because they are undergoing chemotherapy for Leukemia and are a possible Bone Marrow Transplant patient who should have special requirements).!

At my current position, I have had to call Physicians a couple of times when we received orders on patients with a diagnosis that should have required special blood products. Basically, it was me telling them what to order based on the standard of practice.

Brenda Hutson, CLS(ASCP)SBB

I agree with you 100% Brenda about the need for a proper diagnosis.

What does "Pre-op" mean? Fixing an ingrowing toe nail or a heart-lung transplant?????????

:mad::mad::mad::mad::mad:

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Wow! Thank you for your responses. I am assuming that many practices that do address phenotypic needs seem to address mainly Kell and Rh. Is there any litterature that suggests a propencity on part of a patient with Sickle Cell Disease to readily develope antibodies of these two systems over other systems or are we working mainly with a convention in practice? Additionally, I have not heard of the practice to do a saline wash of the red cells before performing a phenotype of recipient cells when they have been transfused within the last three months. What is the theory behind this practice that now makes the results of the procedure more reliable?

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Wow! Thank you for your responses. I am assuming that many practices that do address phenotypic needs seem to address mainly Kell and Rh. Is there any litterature that suggests a propencity on part of a patient with Sickle Cell Disease to readily develope antibodies of these two systems over other systems or are we working mainly with a convention in practice? Additionally, I have not heard of the practice to do a saline wash of the red cells before performing a phenotype of recipient cells when they have been transfused within the last three months. What is the theory behind this practice that now makes the results of the procedure more reliable?

Hi,

I can't answer the second question in your post (regarding washing red cells), but I may be able to help you with the first question.

If you go to "References" at the top of the page and click on it, then click on "Document Library" on the drop-down list, this will take you to another list, one of which is named "Educational Material". If you click on that, you will have another list and in that is an essay that I submitted entitled, "Phenotyped Red Cell Transfusions". In there is a paragraph specific to Sickle Cell Patients, and this will tell you why giving Rh and K matched blood helps to cut down on the production of antibodies. Of course, I am quoting an awful lot of other people's work, and you will find that each paper I quote is cited in the References.

I hope it will go some way to answering your first question.

:):):):):)

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Wow! Thank you for your responses. I am assuming that many practices that do address phenotypic needs seem to address mainly Kell and Rh. Is there any litterature that suggests a propencity on part of a patient with Sickle Cell Disease to readily develope antibodies of these two systems over other systems or are we working mainly with a convention in practice? Additionally, I have not heard of the practice to do a saline wash of the red cells before performing a phenotype of recipient cells when they have been transfused within the last three months. What is the theory behind this practice that now makes the results of the procedure more reliable?

The wash is a hypotonic wash of the recipient cells. Normal red cells do not tolerate the hypotonic solution and will lyse, leaving only the sickle cells behind. Presumably, these cells belong to the patient (although if the transfused units were not sickle negative, I suppose it is possible that some would be left behind). Then you can perform your phenotyping despite the recent transfusion. I am not at work right now, so I don't have references to offer. Perhaps someone else who has their references to hand can help with this...

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Wow! Thank you for your responses. I am assuming that many practices that do address phenotypic needs seem to address mainly Kell and Rh. Is there any litterature that suggests a propencity on part of a patient with Sickle Cell Disease to readily develope antibodies of these two systems over other systems or are we working mainly with a convention in practice? Additionally, I have not heard of the practice to do a saline wash of the red cells before performing a phenotype of recipient cells when they have been transfused within the last three months. What is the theory behind this practice that now makes the results of the procedure more reliable?

The Rh and Kell system Antigens are more antigenic; that is why they start out matching those groups. If you can avoid antibodies to those groups, you may be able to avoid antibodies all together in these patients. And the problem with sickle cell patients is that when they come in, it is often "in crisis." So, you need to set up a scenario where you can get them blood as quickly as possible. That is one of the main reasons you don't want them to make any antibodies; that way, if blood is needed urgently at some point, you can just give them random blood (you may not have to worry about it for that transfusion, but of course you have now set them up to make the Rh and/or Kell system antibbodies). The other thing to keep in mind is that even if you do match for Rh and K, some of these patients have e variants (which you may not detect until they make the antibody).

When non sickle cell patients have been transfused in the past 3 months, you can only get an accurate phenotype by separating the patient from donor cells by reticulocyte separation (the patient's reticulocytes). With sickle cell patients, it is easier than that. The hypotonic wash will destroy the donor cells but leave the patient's sickle cells intact; then you can type those cells. It is just a function of the difference between normal RBCs and sickled cells.

Hope that helps,

Brenda Hutson

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Hi,

I can't answer the second question in your post (regarding washing red cells), but I may be able to help you with the first question.

If you go to "References" at the top of the page and click on it, then click on "Document Library" on the drop-down list, this will take you to another list, one of which is named "Educational Material". If you click on that, you will have another list and in that is an essay that I submitted entitled, "Phenotyped Red Cell Transfusions". In there is a paragraph specific to Sickle Cell Patients, and this will tell you why giving Rh and K matched blood helps to cut down on the production of antibodies. Of course, I am quoting an awful lot of other people's work, and you will find that each paper I quote is cited in the References.

I hope it will go some way to answering your first question.

:):):):):)

At the very grave risk (indeed, almost a certainty) of sounding very big-headed, it might be worth your while following the instructions above, but, instead of clicking on "Phenotyped Red Cell Transfusions", click instead on "Blood Transfusion Therapy for Haemoglobinopathies". This explains it in a bit more detail, and also gives some references to read on the subject.

Sorry to self-advertise everybody.

:redface::redface::redface::redface::redface:

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Thank you all again for your valued responses. I am understanding that the hopotonic solution will lyse normal RBC's and leave behind the patient's Sickle Cells for phenotyping. This is a unique way of developing this distingtion however I wonder how reliable the actual phenotyping of Sickle Cells realy is given the random surface distortion of the rbc membrane and it's effect on the distribution of the very surface antigens we are typing for. My biggest concern here is steric hinderence that will potentially interfere with Ag-Ab reactions such that we may miss a phenotype and interpret a negative when the result was realy positive. Ultimately I wonder if steric hinderence will cause reactivity that falls below the sensitivity of the Ag system we are testing for?

Additionally it is very interesting to see that the Rh and Kell systems are more antigenic than the other systems and further more, that once the immunsystem of this patient is sensitized to either or one of these systems it then has the propencity towards sensitization to the other systems. Are there any noted exceptions in your experiences or literature where a patient with sickle cell disease developed Abs to one of the other systems first?

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52 year old black man was coming in for a transfusion yesterday. He has a history of chronic anemia. No one thought until recently that he could have sickle cell disease. Working him up for the first time after many years of transfusion. I believe his antibody screen was negative, but we did a complete phenotype on him and started following the proper protocol.

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Thank you all again for your valued responses. I am understanding that the hopotonic solution will lyse normal RBC's and leave behind the patient's Sickle Cells for phenotyping. This is a unique way of developing this distingtion however I wonder how reliable the actual phenotyping of Sickle Cells realy is given the random surface distortion of the rbc membrane and it's effect on the distribution of the very surface antigens we are typing for. My biggest concern here is steric hinderence that will potentially interfere with Ag-Ab reactions such that we may miss a phenotype and interpret a negative when the result was realy positive. Ultimately I wonder if steric hinderence will cause reactivity that falls below the sensitivity of the Ag system we are testing for?

Additionally it is very interesting to see that the Rh and Kell systems are more antigenic than the other systems and further more, that once the immunsystem of this patient is sensitized to either or one of these systems it then has the propencity towards sensitization to the other systems. Are there any noted exceptions in your experiences or literature where a patient with sickle cell disease developed Abs to one of the other systems first?

As far as the hypotonic wash, it is used by reference labs everywhere; to my knowledge, it is a reliable test.

My comment about the "Rh and Kell systems being more antigenic and hopefully, the patient would not make other antibodies," was not to infer that somehow matching for Rh and Kell "protected" the patient from making other antibodies. When you work in a reference lab long enough, you realize that even though E and K are not of particular high incidence, they are the most common antibodies you see (because they are more antigenic). So, matching for Rh and Kell does not protect the patient from making other antibodies; it is just that those would be the most common antibodies they would make so you are going with statistics. From a statistical standpoint, the "chances" of them making antibodies to the other groups is not as high as it is for Rh (and keep in mind that most/many SS patients are RoRo so if you give them Rh Positive Blood, you are likely exposing them to at least C and sometimes E).

Then there is the fact that some patients are just better antibody producers than others and they will make antibodies to about everything they can (so while you protected them from making the Rh and Kell group antibodies, they will make others).

One other thing to add to the confusion (in addition to partial e and the difficulty that can cause in transfusing these patients); many/most of these patients will be Fy(a-b-); however, they don't usually make the anti-Fyb (you can read up about that in the literaturer). So, if your patient makes 1 antibody to something such that you are now giving them totally phenotypically matched, you can usually get away with not worrying about giving Fyb-.

Brenda Hutson, CLS(ASCP)SBB

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Thank you all again for your valued responses. I am understanding that the hopotonic solution will lyse normal RBC's and leave behind the patient's Sickle Cells for phenotyping. This is a unique way of developing this distingtion however I wonder how reliable the actual phenotyping of Sickle Cells realy is given the random surface distortion of the rbc membrane and it's effect on the distribution of the very surface antigens we are typing for. My biggest concern here is steric hinderence that will potentially interfere with Ag-Ab reactions such that we may miss a phenotype and interpret a negative when the result was realy positive. Ultimately I wonder if steric hinderence will cause reactivity that falls below the sensitivity of the Ag system we are testing for?

Additionally it is very interesting to see that the Rh and Kell systems are more antigenic than the other systems and further more, that once the immunsystem of this patient is sensitized to either or one of these systems it then has the propencity towards sensitization to the other systems. Are there any noted exceptions in your experiences or literature where a patient with sickle cell disease developed Abs to one of the other systems first?

You theorise upon an interesting concept here. Indeed, of course, steric hinderence is already known about with the ABO group hindering the antigen/antibody reaction between anti-H and H, and the Kell glycoprotein hindering the reaction between anti-Kx and Kx, so, in theory at least, this could happen with the distortion of the sickle cell. However, I think that the distortion is at a gross level, rather than at a microscopic level, and so the antigens, so long as they are evenly spread around the cell surface, and they are numerically high, should still be "available" - but I don't know that for sure.

Yes, there are plenty of cases of sickle cell patients making antibody specificities without first making Rh or K antibodies (I think I mentioned one in a post the other day who had made anti-S+Fya+Fy3+Jka, which kept me up most of the night!).

By the way, have you noticed how many sicklers who go on to make anti-Fy3 seem to produce an anti-Fya (or apparent anti-Fya) first, if you "catch them early enough"?

:confused::confused::confused::confused::confused:

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  • 2 weeks later...

Dear Malcolm,

I have not ever seen a sickle cell patient with Anti-Fy3 and had it not been for you metioning it here I would not have heard of it either. Upon reviewing the AABB Technical Manuel I see that there indeed is a Fy3 which I initially thought was another name for Fyb. Interestingly, I came to see that the Duffy antigen is also a genetic combatent of malarial infestation, the same as the sickle cell. If the sickle cell patient produces anti-Fy3 and anti Fya would they necessarily antigen type Fyb positive?

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