Lutheran Blood Group System Flashcards

1
Q

Talk about the clinical significance of the lutheran blood group

A

The Lua antbiody is the main antibody we see
Lua is not clinically significant
The lub antibody is rarely seen but Lub is clinically significant
The Lu3 antibody is seen in the null state

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Talk about the discovery of the Lutheran blood group

A

It was originally discoveed in a blood donor

It was the fifth discovered blood group

It is mispronounced -> Should be luth-er-an not lootheran but the name has stuck

Anti-Lua was discovered in 1945 in a serum that contained several other antibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Talk about the genetics of the lutheran system

A

It is a single locus system
It consists of 25 antigens
The genes of the Lutheran group are linked to the genes responsible for the secretion of ABH substances i.e. the Se gene on chromosome 19
The main antigens are Lua and Lub
The majoriity of antigens are purely academic and we dont see them in clinical setting

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the function of the Lu blood group system?

A

It helps erythrocytes locate to stromal cells of bone marrow

Haematopoiesis will occur more distriubted in the body if you are missing the Lutheran antigen

It will occur outside of the bone marrow without lutheran

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Talk about the lutheran antibodies

A

It has an IgM and IgG component

Anti-Lua was the first discovered antibody in 1945 in serum containing several other antibodies

i.e not high sensitisation rate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Talk about the frequency of the Lu antigens

A

Lu (a+b+) 7.5
Lu (a+b-) 0.15
Lu (a-b+) 92.35
Lu (a-b-) Very rare

NB: >99% are Lub+

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

How does Lua and Lub differ from each other?

A

There is a single base pair change difference
His 77 Arg

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is a good way of remembering the frequencing of the Lua antigen?

A

There is always one Lua positive cell in an antibody panel i.e. 1/12 cells
Lua frequency = 1/12 or approximately 8% (7.65% exactly)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Talk about the lutheran antibodies

A

igG/IgM
They are not enzyme sensitive
Not often encountered especially anti-Lub
There is often variation in Lu antigen strength
Crossmatch compatible blood issues

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the other important antigens of the Lu system?

A

Auberger antigens:
Au(a) = 80% of population
Au(b) = 50% of population

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the telltale signs of anti-lutheran antibodies?

A

They tend to give clear reactions in gels - normal reactions

Tend to give scruffy reactions in tubes - these dont look like aggregation, looks more like clotting, looked different under the mic as well

Scientist often able to do this - how they used to be identified - characteristic clumping

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Talk about anti-Lua

A

IgM and IgG (mostly IgM though)
Usually not clinically significant in transfusion but mild cases of HDFN have been reported (igG type)
Anti-Lua may be present without immune red cell stimulation
Optimal in vitro agglutination reactions are observed at room temperature but gives a characteristic mixed-field/scruffy pattern of agglutination

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Why does Lua not cause clinically significant reactions?

A

There are very few copies of the antigen on the red cells - only a few thousand copies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Talk about anti-Lub

A

Rare antibody due to the high incidence of Lub >99%
IgG class
Most anti-Lubs agglutinate at the antiglobulin phase but some examples of Lub show a mixed-field agglutination pattern
Anti-Lub has been associated with transfusion reactions and mild cases of HDFN
Fabian has never seen an anti-Lub

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Why does Lua cause mixed-field reactions?

A

Small agglutinates are surrounded by unagglutinated free red cells

Few antigens on red cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How does anti-Lua compare to anti-Lub?

A

Lub is a more typical antibody while Lua tends to be more IgM, cold reactive etc

17
Q

Talk about Lu(a-b-)

A

Very rare but may arise from genetic circumstances
- amorphic lutheran gene (LU) inherited from both parents
- In (LU), an inhibitor gene which prevents normal expression of Lutheran
- Suppressor gene XS2

18
Q

Talk about the lutheran inhibitor In (Lu))

A

A dominant trait attributed to an inhibitor gene which prevents the normal expression of Lutheran and certain other blood group antigens, notably P1, I, In (a) and In (b)

19
Q

How prevalent is the In (Lu) phenotype, inhibitor?

A

It has a prevalence of 0.03%

20
Q

How is In (Lu) expressed

A

It affects GPIs on the surface of cells -> it affects IKAMS like Lutheran and Indian blood group system

It reduces expression of these antigens
- these wont produce an anti-Lu3 -> these are not true Lu negative

21
Q

Talk about amorphic lutheran gene (LU)

A

This is the same as RhNull
It is usually familial, happens when two cousins married etc etc
The only kind of true Lu-
These will produce an anti-Lu3

22
Q

Talk about amorphic lutheran gene (LU)

A

This is the same as RhNull
It is usually familial, happens when two cousins married etc etc
The only kind of true Lu-
These will produce an anti-Lu3

23
Q

Talk about the XS2 gene

A

An X-borne suppressor gene XS2
It is recessive in its effect -> males only affected
Found on the x chromosome

24
Q

Where is the LU locus found?

A

Amorphic lutheran gene LU is found on chromosome 19

25
Q

Talk about the lutheran antigens

A

600-3800 copies per red cell

The lutheran glycoprotein is an adhesion molecule which binds to laminin

The antigens are destroyed by trypsin, a-chromotrypsin and sulfinhydryl-reducing agents

26
Q

What destroys the lutheran antigens

A

Trypsin
a-chromotrypsin
Sulfinhydryl-reducing agents

NB: sulfinhydryl is the only one still used

27
Q

What is sulfhydryl-reducing agents, how do we use these?

A

This is what is used in DTT treatment

DTT for treatment of daratumumab

Its also found in ZAP used in adsorptions

If you query an Lub you can use DTT to treat cells and all reactivty will disappear