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Blood Group Antigen

Genetically encoded antigens on the red cells, white blood cells and platelets
Classified as:
Carbohydrate antigens/ Protein antigen
Low incidence/ high incidence

Blood Group Terminology:

Genes written in ITALICS/ UNDERLINED, number is SUPERSCRIPT; Ex A1


Antigens written in REGULAR type; Ex. A1
Phenotype allele number is SUBSCCRIPT; Ex. A1

LEWIS SYSTEM
Lewis gene is found on chromosome 19
Distantly linked to Se and H genes
90% of white population possesses Le gene
Lewis antigens are secondarily absorbed to the red cells
Le gene produces Lea substance
Secretors change the Lea to Leb

I. Le(a+b-)Non-secretors
Lea substance are secreted regardless of the secretor status.
Non-secretors

II. Le(a-b+)Secretors
Leb is a product of genetic interaction (Se and Le)
ABH secretors, Lea and Leb secretors

III. Le(a-b-)
Le(a-b-) secretors is type 1 H(Led)substance
Le(a-b-) Non- secretors is type 1 (Lec)
80% ABH secretors, 20% ABH non secretors

IV. Le(a+b+) Partial secretor


Weak variant of secretory gene is responsible Sew
Reported more on group O individuals

Development and changes of Lewis antigens in newborn:


1. ABH substances are present in saliva of newborn infants.
2. Plasma- no detectable Lewis glycosphingolipids
3. Cord blood sample phenotype as Le(a-b-)

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4. Le and sese genotype, antigens are not detectable on cord RBC but secrete Le a in
saliva.
5. Le and Se genotype
6. from Le(a-b-) at birth to Le(a+b-)after 10 days to Le(a+b+), and finally Le(a-b+).
7. lele genes phenotype as Le(a-b-) for the rest of their life
8.

Lewis Antibodies
Anti-Le a, Anti-Le b, Anti-Lex - generally produced by Le(a-b-)
Reactive with enzyme treated cells

I. Anti-Le a
Most common
Can be IgG partly or entirely
Le(a-b+) do not make Anti-Lea
II. Anti-Le b
Usually produced by Le(a-b-)
Often found with Anti-Lea
Most react at room temperature or below
Two types - Anti-LebH and Anti-LebL
III. Anti-Lex
Reacts with both Le(a+b-) and Le(a-b+) RBCs
Formed by Le(a-b-) individuals

IMPORTANT:

Leb - associated with receptor of H.pylori and Norwalk virus


Lex marker for Reedsternberg cell
Le (a-b-) increase renal graft and heart disease; increased susceptibility to
infections by Candida and uropathogenic E.coli

I BLOOD GROUP SYSTEM


I stands for Individuality
Rare adult i
Ii are enhance by enzymes
I and i antigens are found on the membranes of leukocytes and platelets in
addition to RBCs
HEMPAS has greater i activity on RBCs than control cord RBC

IT Antigen and Antibody


Does not demonstrate classical I or i
Transition state of i into I

I Antibodies

I. Anti-I
COLD AUTOAGGLUTININ
Reacts at room temperature
Testing at 4 degrees or against enzyme treated RBCs
Binds complement
II. Pathogenic Anti-I
consist of strong Cold agglutinin syndrome
reacting up to 30 to 32 degrees

I Antigens
Two biosynthetic pathway and genes are involved in development and expression.
1. P1 and Pk antigen is assigned to the P1PK blood group system.

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2. P antigen is assigned to the Globoside blood group system (028). LKE and
PX2 (209)
P BLOOD GROUP SYSTEM

P Antibodies

I. Anti P1
naturally occurring IgM
Found in patients with Fascioliasis
II. Anti PP1Pk (Formerly Known Anti Tja)
Increased incidence of spontaneous abortion
III. Alloanti P
associated with habitual early abortion
IV. Autoanti P
cold-reactive IgG autoantibody (Biphasic Hemolysin)
PCH
V. Anti-Pk
Anti-Pk has only been found as part of other antibodies
Activity can be inhibited by hydatid cyst fluid
VI. Luke(LKE) Antigen and Antibody
Described in serum of patients with Hodgkins lymphoma.
All individual with p and Pk phenotype are Luke.

Disease Association:
1. Anti P1 = associated with parasitic infections
2. Anti PP1k = associated to spontaneous abortion
3. Anti P = PCH
4. P system antigen = serve as receptors for P-fimbriated uropathogenic E. coli
5. Pk antigen = receptor for shiga toxins
6. P antigen = receptor of parvovirus B19

DUFFY BLOOD GROUP SYSTEM

Duffy Antibodies

Anti-Fya Anti-Fyb
Usually IgG
Rare examples of antibodies to other antigens (Anti-Fy 3, Anti-Fy4, Anti-Fy5) and
those reactions are not destroyed by enzymes

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The Kell (006) and Kx (019) Systems

Antigens are denatured by the following except Kx:


Trypsin and chymotrypsin in combination
DTT, 2 mercaptoethanol
AET and ZZAP
Glycine acid-EDTA (IgG removal agent)

Except by ficin and papain

Kx is present on all RBCs except those of the rare Mcleod phenotype


K0 is a null phenotype
o Immunized individuals produce anti-Ku( K5) which recognize all Kell antigens

Mc Leod phenotype and syndrome


Reduced expression of Kell antigens
association with CGD
Associated with mutations and deletions at the XK locus

Antibodies

I. Anti-K
A very common antibody encountered in blood bank
Made in response to antigen exposure through pregnancy or
transfusion
Most reliable method of detection is IAT

MNSs Blood Group

Many antigens in this system and some are alleles to the four common antigens
M N- found in GPA
Amino acid residue at positions 1 and 5
- M has serine and glycine
- N has leucine and glutamic acid
- Easily destroyed by enzymes
- Not affected by DTT alone

Antibodies:

I. Anti-M
Dosage (antibodies react better with homozygous cells)
Possible HDN and HTR if reacts w/ AHG

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Reacts best at ph 6.5
II. Anti-N
Cold reactive IgM or IgG saline agglutinin
Anti-Nf found in dialysis patients
Most potent antibodies are found in individuals who type as M+N-S-s-
III. S and s Found in glycophorin B
S Has methionine whereas s has threonine
Less easily degraded by enzymes
IV. Anti-S and Anti-s
Most examples are IgG, reactive at 37C and AHG
HTR and HDN

U antigen is absent or reduced on S-s-


Mi - abnormal forms of Ss glycoprotein
En(a-) absence of MN glycoprotein

GPA and GPB-deficient Phenotypes:


I. U-Phenotype
U for universal antigens present in GPB
Type as S-s-U- and make Anti-U in response to transfusion or pregnancy.
II. En(a-)
No GPA produced, but GPB is not affected. This type of En(a-) inheritance is called
En(a-)Fin
III. Mk Phenotype
Is the null phenotype in the MNS system
MkMk genotype

The Kidd (009) System

Jka and Jkb Antigens


Detected on fetal red cells as early as 11 weeks for Jka and 7 weeks for Jkb

Antibodies:
I. Anti-Jka and Anti-Jkb
Usually IgG and require AHG
bind complement
Classic delayed HTR
Have notorious reputation in the blood bank
II. Anti-Jk3
found in some Jk(a-b-) individuals
reacts with Jka and Jkb

The Lutheran (005) System

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Antibodies:

I. Anti-Lua - not common - reacts in saline but can be IgG and require AHG
II. Anti-Lub - rare - mostly IgG and requires AHG
III. Anti-Luab (Anti-Lu3 ) produced by Lu(a-b-)

The Diego (010) System


Antigens: Dia/Dib, Wra/Wrb, and Wu/DISK
Dia = marker of Mongoloid ancestry
Diego antibodies: Usually IgG

The Yt (011) System

Yta :high-prevalence antigen in all populations


Ytb : low-prevalence antigen
Antibodies: usually IgG (cant cause HDFN)

The Xg (012) System


Controlled by X linked gene
Antigen has a higher prevalence in females than in males
Two antigens: Xga and CD99

The Scianna (013) System


High prevalence antigen: Sm (Sc1)
Low prevalence: Bua (Sc2)

The Dombrock (014) System


Dombrock antigens :carried on a mono-ADPribosyltransferase 4 (ART4) attached
to the RBC membrane by a GPI anchor.
The Doa and Dob antigens :poor immunogens
Gya, :highly immunogenic.

The Dombrock (014) System


The high- and low-prevalence antithetical antigens are Coa and Cob, respectively
The Colton antigens :carried on an integral membrane protein, aquaporin 1
(AQP1)

The Landsteiner-Wiener (016) System

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The Chido/Rodgers (017) System

Not intrinsic to the RBC membrane


Neutralized by the plasma

The Gerbich (020) System

The Cromer (021) System


Antigens are carried by the DAF (CD55)

The Gerbich (020) System


Antigens are located on Complement Receptor 1 (CR1)

Other blood group

I. The Indian (023) System


II. The Ok (024) System
III. The Raph (025) System
IV. The John Milton Hagen (026) System
V. The Gill (029) System
VI. The RH-Associated Glycoprotein (030) System
Miscellaneous antigens:
I. Vel
II. Ata
III. Sda
a. Found in: saliva, urine, and meconium

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