Transfusion Reaction caused by Redelberger

[jayinsat]

Here is a case that I found interesting a few years ago:

A 77 y.o. male patient presented for transfusion. Pretransfusion study demonstrated a B positive, antibody screen negative individual. B positive units were crossmatched, immediate spin in tube, and found to be compatible. One unit was issued and transfused to the patient. Two hours into the transfusion the patient began experiencing symptoms of an acute transfusion reaction which included fever, chills, pain in lower back and neck, tachypnea, and hypertension. Transfusion was stopped, however, the patient had received 250 cc's of the unit. The unit was returned to the blood bank for transfusion reaction workup, along with a new sample.

The post transfusion reaction specimen was significantly icteric as compared to the pretransfusion sample. There were no clerical errors and the retyping of both the pre and post sample yeilded the same results; B positive with a negative antibody screen. The direct coombs on the post sample was positive with Polyspecific and IgG monoclonal antihuman globulin and negative with C3b. The corresponding elution yielded all cells negative with an Ortho 0.8% gel panel. The crossmatch was compatible at immediate spin with both the pre and post samples but 2+ incompatible at coombs (on gel). An Immucor 16cell tube panel was run and the patient's plasma reacted positive for 1 of the 16 cells.

The reacting cell was uniquely positive for the Redelberger A antigen. As no other reagent red cells with Redelberger A were available for testing, the specimen was sent to the American Red Cross for further identification and confirmation of the unidentified, low frequency antibody. Units compatible through coombs were selected and transfused uneventfully.

The follow-up report from ARC revealed that the unidentified antibody was anti-Redelberger A as well as Diego A. Since the reagent red cell panels (both 16cell and 11 cell 0.8%) were all negative for DiA, it was not originally suspected. Both Redelberger and Diego are low frequency antigens, compatible units should be easy to find. The patients records were marked and future crossmatches will always be performed at both immediate spin and gel-coombs.

[Malcolm Needs ☆]

This is an interesting case indeed.

It is known that, once a person makes an antibody against one low frequency antigen, they tend to make a mixture of antibodies against a host of low frequency antigens (well, perhaps not a host, but certainly there is often a mixture).

It is also known that anti-Dia can be clinically significant.

The interesting thing is that, as far as I know, there has never been a record of anti-Rba being clinically significant (although, being part of the Diego Blood Group System, it was always thought likely to be so).

Was an eluate ever made from the gentleman's post-transfusion sample to prove that the causative antibody was indeed anti-Rba? Unless this was done, it could possibly have been due to yet another antibody directed against a low incidence antigen.

If it was, and anti-Rba was proved to be the causitive antibody, was it ever reported as a case study (or something similar) as this would be a very important piece of evidence that anti-Rba can indeed be clinically significant?

Nice case!

[jayinsat]

This is an interesting case indeed.

It is known that, once a person makes an antibody against one low frequency antigen, they tend to make a mixture of antibodies against a host of low frequency antigens (well, perhaps not a host, but certainly there is often a mixture).

It is also known that anti-Dia can be clinically significant.

The interesting thing is that, as far as I know, there has never been a record of anti-Rba being clinically significant (although, being part of the Diego Blood Group System, it was always thought likely to be so).

Was an eluate ever made from the gentleman's post-transfusion sample to prove that the causative antibody was indeed anti-Rba? Unless this was done, it could possibly have been due to yet another antibody directed against a low incidence antigen.

If it was, and anti-Rba was proved to be the causitive antibody, was it ever reported as a case study (or something similar) as this would be a very important piece of evidence that anti-Rba can indeed be clinically significant?

Nice case!

Thanks Malcolm. If I remember right, the elution I performed on the post-sample reacted against the same Rb cell that we had on the Immucor 16 cell panel. Just like the original neat plasma and pre transfusion sample, it was the only cell that reacted.

The Red Cross did ask permission of our facility to use it as a case study. Also, I believe that they found that there was some sort of familial connection between the donor of the cell that was positive for Rb and the unit of blood implicated in the transfusion. They both traced back to eastern Europe, I believe. (its been a while and my memory is not the greatest)

[Malcolm Needs ☆]

Thanks for that.

Talking of families, I remember Joyce Poole at the IBGRL telling me about a low frequency antigen that she found on the red cells of a sailor from The Netherlands. They did a family study, but also found that he had other "wives" at almost every port that he visited on a regular basis. Needless to say, the low incidence antigen could be traced almost all the way around the world, but for some reason, only at ports!!!!!!!!

:D:D:D:D

[TimOz]

Hi Jayinsat,

Good case.

Just one point (I am pushing a wheelbarrow here) is that Dia (an a few other low incidence antigens) are only low incidence antigens in Caucasian populations. It is not low incidence in many populations like the Japanese, Native American Indians, South Americans with native blood etc. The incidence is not actually characterised in many Asian populations. Maybe it came south with the Mongol hordes and if so will be around quite a bit - and not just in ports ;-)

I find the clinical relevance of Diego interesting as most text books tend to quote that they sometimes cause serious HDFN but not transfusion reactions. I feel this is likely not to be true. It is just that they are not identified. A quick search brings up quite a few recently published cases of Diego antibodies causing serious haemolytic transfusion reactions. Often by the Japanese as they have the screening cells to find them and ID cell to ID them. When you go to the Philippines they get ~30% of antibodies as "unidentified". In Malaysia it is ~24%. These are antibodies found in a crossmatch and are neg on a Caucasian based screen or ID panel. What are they? I am betting quite a few are Di.

Any information on donor source of the Dia and the ethnicity?

Malcolm, your story reminds me of the variegate porphyria found in West Australian Aboriginals that may relate to a specific Dutch family who may have had an ancestor on the VOC ship Zuytdorpt that may or may not have been shipwrecked on the Central WA coast. The disease is found in WA and also South Africa in the Boers (Dutch descent). A more recnet study showed that the Aboriginal version is due to an independant mutation different from the common Dutch mutation. Still makes a good story.

[Malcolm Needs ☆]

Hi Tim,

There is extremely good evidence that the DIA gene did indeed spread west with the invasion of the Mongol hordes. There is DNA evidence for this.

I am at work at the moment (sad, I know - not least for the patients), but I'll try and find the reference when I get home tonight or over the weekend.

[Malcolm Needs ☆]

Right, the reference is:

Zerjal T, Xue Y, Bertorelle G, Wells RS, Bao W, Zhu S, Qamar R, Ayub Q, Mohyuddin A, Fu S, Li P, Yuldasheve N, Ruzibakeiv R, Xu J, Shu Q, Du R, Yang H, Hurles ME, Robinson E, Gerelsaikhan T, Dashnyam B, Mehdi SQ, Tyler-Smith C. The genetic legacy of the Mongols. Am J Hum Genet 2003; 72 (3): 717-721.

The paper actually talks about the results of a study concerning the Y-chromosome, but, as the DIA gene is "common" within Mongolia, the extension is, I feel, justifiable.

:):)

[Yanxia]

Was an eluate ever made from the gentleman's post-transfusion sample to prove that the causative antibody was indeed anti-Rba?

I don't think there is relationship between the binding and the clinical significance of the antibody. Some antibodies can bind to rbc but no ducuction of the cells' life span.

[Malcolm Needs ☆]

I don't think there is relationship between the binding and the clinical significance of the antibody. Some antibodies can bind to rbc but no ducuction of the cells' life span.

No, I realise that shily!

In this case the patient appears to have undergone a fairly convincing acute haemolytic transfusion reaction, from the description of the symptoms he showed.

The point I was making was that if anti-Rba could not be eluted from the patient's red cells, then the transfusion reaction could not have been caused by the anti-Rba, and must have been caused by another, unknown antibody (also, in all probability, directed against a low incidence antigen of unknown specificity).

If anti-Rba could be eluted from the patient's red cells, it does not prove that the anti-Rba caused the transfusion reaction (it could have been another antibody, also, in all probability, directed against a low incidence antigen of unknown specificity - it could not have been the anti-Dia as, as far as I can make out, anti-Dia was not eluted from the patient's red cells). It does, however, strongly suggest that the anti-Rba was involved, as, if this was not involved, the patient's red cells must have expressed at least two different antigens of low incidence - which would not be unique, but would be highly unusual.

:):)

[jayinsat]

I really appreciate the discussions on BBT! I have found tons of useful info just reading comments and even arguments between some very intelligent and knowledgeable blood bankers. Thanks everyone. This forum is truly a hidden jewel!

:):)

[Malcolm Needs ☆]

I really appreciate the discussions on BBT! I have found tons of useful info just reading comments and even arguments between some very intelligent and knowledgeable blood bankers. Thanks everyone. This forum is truly a hidden jewel!

:):)

You know, nobody has called me part of a hidden jewel before.

Many, on the other hand, have suggested I should be buried under a pile of boulders!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!

:eek::eek: