Other Blood Groups 1 Mazen

Other Blood
Groups
Kell, Duffy, Kidd,
Ii, MNSs & P
1
The Kell Blood Group

System
M. Zaharna Blood Bank 2009
Background
information
The Kell blood group system was

discovered in 1946.
Number of Kell antigens: > 20
These antigens are the third most potent,

after those of the ABO and Rh blood
groups, at triggering an immune reaction.
Molecular information
The KEL gene is found on chromosome 7
The KEL gene is highly polymorphic, with

different alleles at this locus encoding the
25 antigens that define the Kell blood
group.
The Kell protein is a polypeptide chain of

732 amino acids in length that becomes
glycosylated at five different sites. It
makes a single pass through the RBC
membrane.
Kell Blood Group System
XK gene produces Kx substance, which is a

precursor of of Kell Ags
Kel genes convert Kx substance into the Kell Ags

on RBCs
K (Kell) & k (cellano) are produced by allelic

genes, this results into 3 phenotypes:
K+k- (genotype KK)

K+k+ (genotype Kk)
K-k+ (genotype kk)
Other allelic genes include: Kpa/Kpb, Jsa/Jsb
XK Gene (Chromosome X)
KEL Gene
RBC
Kx
Kell system glycoprotein:

Kell Ags reside here.
Frequency of Kell phenotypes

Phenotype
Caucasians
Blacks
K-k+
91 %
98 %
K+k-
0.2 %
Rare
K+k+
8.8
Kx Substance
Kx substance is present on RBCs & WBCs
Kell genes convert Kx substance into the Kell Ags

on RBCs
Kell genes do not convert Kx on WBCs
McLeod Phenotype
Absence of Kx proteins in
RBCs membrane lead to
McLeod Phenotype
This absence cause:
abnormal RBCs shape

(acanthocytes)
& reduced in-vivo survival
Chronic Granulomatous
Disease
Absence of Kx proteins in WBCs cause

CGD
Leukocytes are able to phagocytose but

not to kill bacteria
Patients with CGD have recurrent bacterial

infections
Patients who lack Kx on RBCs & WBCs

have both Mcleod and CGD
Kell Antibodies
K- individuals produce anti-K when

exposed to K+ cells
Frequency of K+ is low (9%), easy to find

blood
On the other hand frequency of k is 99.9%
k- individuals produce anti-k when exposed to

k+ cells
Difficult to find blood
Antibodies produced against

Kell antigens
Kell Abs
Clinically
Significant
Abs class
Yes
IgG (rarely) IgM
Thermal range
HDNB
4 - 37
Yes
Transfusion Reactions
Extravascular
Intravascular
Yes
Rare
Duffy Blood Group

System
Duffy Blood Group System
The Duffy blood group was discovered in 1950.
The Duffy glycoprotein is encoded by the FY gene,

found on chromosome 1 , of which there are two
main alleles, FYA and FYB. They are codominant.
The Duffy gene codes for a glycoprotein also found

in other tissues: brain, kidney, spleen, heart and
lung.
The Duffy glycoprotein is a transmembrane protein
Five alleles at Duffy locus, the most important: Fya,

Fyb & Fy (Silent Allele)
Fya is more immunogenic than Fyb

Duffy Antigens
Phenotype Frequencies
Blacks
Phenotype
Caucasians %
Fy (a+b+)
49
Fy (a+b-)
18
14
Fy (a-b+)
33
19
Fy (a-b-)
rare
65
Different genes
Fy(a-b-) blacks do not produce anti-Fya or

anti-Fyb following transfusion with Fy(a+)
or Fy(b+) blood
Fy(a-b-) Caucasians become sensitized

following transfusion with Fy(a+) or
Fy(b+) blood
This suggest that Fy(a-b-) phenotype

arises from different genes in the two
populations
Duffy Antigens
Fya, Fyb antigens are Destroyed by enzymes
Abs DO NOT agglutinate enzyme treated cells
Moderately immunogenic
Fya is more immunogenic than Fyb
Duffy Antibodies
IgG antibodies and

can activate
complement
Anti- Fya is more
frequently encountered
Anti- Fyb is more

frequently found in
patients produced
multiple alloantibodies
Duffy Abs
Clinically
Significant
Abs class
Yes
IgG
Thermal range
HDNB
4 - 37
Yes
Extravascular
Intravascular
Yes
Yes
Duffy and Malaria
Black people with the Duffy phenotype of Fy(ab)

appear to have resistance to Plasmodium vivax &
Plasmodium knowlesi causative agents of Malaria.
Duffy antigens appear to be a receptor for the P. vivax

organism and when the antigen is not present on the red
blood cell membrane P. vivax is unable to access the red
blood cell
Some areas of West Africa are 100% Fy(ab).
Plasmodium falciparum binds to RBCs at integral

glycophorin A & B
Kidd Blood Group System
Kidd Blood Group System
The Kidd blood group was discovered in 1950.
The Kidd gene is located on chromosome 18
Three alleles: Jka, Jkb, Jk
Codominant Inheritance
Jk is a silent allele (amorph)
The Kidd protein is an integral protein of the RBC

membrane.
Kidd Phenotype Frequencies

Phenotype
Caucasians (%)
Jk (a+ b-)
29
Jk (a+ b+)
49
Jk (a- b+)
22
Jk (a- b-)
Exceedingly rare
Phenotype Frequencies
What is the purpose of learning the phenotype
frequencies of each blood group antigen?
When crossmatches are required it helps the Tech know

how many units to crossmatch or antigen type to find
compatible blood.
If a patient has anti-Jka antibody how many RBC units need to

be antigen typed to find 2 compatible units?
78% of the population is positive for the antigen therefore 22%
are NEGATIVE for the antigen. Approximately 2 out of 10
units are compatible. Need to antigen type 10 units.
Kidd Antigens & Antibodies
Ags are well developed at birth
Have tendency to drop to low or undetectable levels

following formation.
Abs are of IgG type & can activate complement

(Anti-Jka, Anti-Jkb )
Produced following transfusion or pregnancy
Can cause HDNB
They are also a very common cause of delayed HTRs
Ii Blood Group
Found nearly on all RBCS
Their products are transferase enzymes

that attach repeating units of Gal and
GlcNAc to the ABO Precursor Substance.
Big I gene codes for branching of the

Precursor Substance.
Ii Antigens
Little i antigen is LINEAR
Big I antigen is BRANCHED
Found on cord cells, predominantly

Gradually convert from i to I during the
first 18 months of life. Not all i converted
to I, some i still present on adult cells,
normally.
Rare adult individuals termed iadult do

not express i Ag on their red cells
The I and i antigen sites are considered

uncompleted ABH active chains.
When ABH are removed from RBCs more I Ags

are expressed
I structure located beneath the ABH Ags

I Antibodies: Anti-I
Anti-I is naturally
occurring often due to a
Mycoplasma pneumoniae
infection
Anti-I reacts with all
adult cells (including
patients own, all reagent
cells, all donor cells)
Anti-I does not react
with cord cells
Auto-anti-I is a common
cold agglutinin
Anti-I Abs
Clinically
Significant
Abs class
Rare
IgM
Thermal
range
HDNB
4 - 10
No
Extravascular
Intravascular
No
rare
Antii Antibodies
Antii
is rarely found in healthy

individuals
Reacts preferably with cord cells
anti-i can be found secondary to

Infectious Mononucleosis.
Transient: Only present with active disease
MNSs Blood Group System
The antigens M and N are produced by

co-dominant alleles
closely linked to the S and s genes,

which are also co-dominant.
Chromosome 4 contains these linked

genes
Genes produce two distinct

glycophorins or sialyglycoproteins
(SGP) on the RBC membrane.
MN Genetics
MN Locus genes produce Glycophorin A (GPA)
M-GPAs 1st five aas = Serine-Ser-Thr-Thr-Glycine
N-GPAs 1st five aas = Leucine-Ser-Thr-Thr-Glutamic

acid
Amino acids (aa) 2, 3 & 4 are the same for both
Glycophorin A (GPA) is a glycoprotein also known as

MN-sialoglycoprotein
MN Genotypes &
Phenotypes
Phenotype
Genotype
Frequency %
M+N-
MM
30
M+N+
MN
50
M-N+
NN
20
MNSs Antigens
M
Glycophorin A
M & N only differ in

their amino acid
sequence at positions
1 and 5
RBC
Glycophorin B
COOH end ..
.5, 4, 3, 2, 1 (NH2 end)

S & s only differ in

their amino acid
sequence at position
29
Ss Genetics
Ss genes code for the production of
Glycophorin B(GPB)
S glycophorin B has Methionine at aa
position 29
s glycophorin B has Threonine at aa
position 29
Glycophorin B (GPB) is a glycoprotein also
known as Ss-sialoglyprotein
Ss Genotypes & Phenotypes

Phenotype
Genotype
S+s-
Frequency %
Caucasians
Blacks
SS
11
S+s+
Ss
44
24
S-s+
ss
45
68
S-s-
S usu
U antigen is a high incident antigen NOT seen in individuals

who lack both S and s antigens.
Individuals who lack this antigen (<1%) have a high likelihood
of forming anti-U as well as anti-S and anti-s.
Rare Alleles
Rare low incidence alleles found on MN

locus
Some may result from crossing over of

genes of glycophorin A & B
Such crossing over results in hybrid

sialoglycoproteins
Anti-M Antibodies
Anti-M Abs
Variability of reactivity
(Dosage)
Strong reactions with

RBCs homozygous for
MM
Weak reactions with
RBCs heterozygous MN
Clinically
Significant
Abs class
Seldom
IgG & IgM
Thermal
range
HDNB
4 22
rare
Rare 22-37
Extravascular
Intravascular
Rare
No
Anti-N antibodies
Anti-N Abs
Naturally occurring
cold agglutinin
Can form in patients
with renal Failure
During dialysis with
formaldehyde
sterilized equipment
Formaldehyde may
alter the N Ag
structure making it
appear foreign
Clinically
Significant
Abs class
IgM
No
Thermal
range
HDNB
No
4 - 22
Extravascular Intravascular
No
No
Anti-S and Anti-s antibodies

Anti-S Abs
Clinically
Significant
Anti-s Abs
Abs class
IgG & IgM
Sometimes
Thermal
range
Clinically
Significant
Abs class
IgG
Yes
HDNB
Yes
4 - 37
Thermal
range
HDNB
Yes
4 - 37
Extravascul
ar
Intravascula
r
Extravascul
ar
Intravascula
r
Yes
No
Yes
No
P Blood Group System
Genetics: These genes code for enzymes

that sequentially add sugars to precursor
substance.
This system is related to the ABO, Le and

Ii systems.
Genes: P1, Pk, P and lower case p (silent

allele)
All antigens are expressed on glycolipids

on red cells
Phenotypes, Detectable Antigens &

Frequencies
Phenotype
Detectable Frequencies
Antigens
Whites %
P1
P1, P
79%
P2
21%
P k1
P, Pk
Rare
P k2
Pk
Rare
N/A
Rare
Pk is the precursor of P.
Rare individuals do not convert Pk into P.
Those will have Pk on RBCs.
Anti-P1 Antibodies
Naturally occcurring
Abs found in the
serum of P2
Individuals
Anti-P1 Abs
Clinically
Significant
Abs class
IgM
occasionally
Thermal
range
HDNB
Yes
4 22
Rare 22-37
Extravascular
Intravascular
No
Rare
Allo Anti-P Antibodies

Naturally occcurring
Abs found in the
serum of Pk and p
Individuals
Allo Anti-P Abs

Clinically
Significant
Abs class
Yes
Rare IgG
Thermal
range
HDNB
IgM
Rare
4 37S
Extravascular
Intravascular
No
Yes
Auto anti-P Antibodies
It is an IgG biphasic Ab
associated with Paroxysmal
Cold Hemoglobinuria (PCH)
Binds complement at cold

temperatures and activates
that complement in warm
temperatures lysing the red
blood cells.
Auto Anti-P Abs

Clinically
Significant
Abs class
IgG
Yes
Biphasic
HDNB
Binds at 0
Rare
Hemolysis 37
Extravascular
Intravascular
Rare
Yes
Anti Tja Antibodies
Combination of anti-P, anti-P1 & anti-Pk
Found in serum of individuals who have no P, P1

& Pk Ags on red cells

Other Blood Groups 1 Mazen

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Other Blood

The Kell Blood Group

M. Zaharna Blood Bank 2009

The Kell blood group system was

Number of Kell antigens: > 20

These antigens are the third most potent,

M. Zaharna Blood Bank 2009

The KEL gene is found on chromosome 7

The KEL gene is highly polymorphic, with

The Kell protein is a polypeptide chain of

M. Zaharna Blood Bank 2009

Kell Blood Group System

XK gene produces Kx substance, which is a

Kel genes convert Kx substance into the Kell Ags

K (Kell) & k (cellano) are produced by allelic

K+k- (genotype KK)

Other allelic genes include: Kpa/Kpb, Jsa/Jsb

M. Zaharna Blood Bank 2009

Kell system glycoprotein:

M. Zaharna Blood Bank 2009

Frequency of Kell phenotypes

M. Zaharna Blood Bank 2009

Kx substance is present on RBCs & WBCs

Kell genes convert Kx substance into the Kell Ags

Kell genes do not convert Kx on WBCs

M. Zaharna Blood Bank 2009

This absence cause:

abnormal RBCs shape

M. Zaharna Blood Bank 2009

Absence of Kx proteins in WBCs cause

Leukocytes are able to phagocytose but

Patients with CGD have recurrent bacterial

Patients who lack Kx on RBCs & WBCs

M. Zaharna Blood Bank 2009

K- individuals produce anti-K when

Frequency of K+ is low (9%), easy to find

On the other hand frequency of k is 99.9%

k- individuals produce anti-k when exposed to

Difficult to find blood

M. Zaharna Blood Bank 2009

Antibodies produced against

IgG (rarely) IgM

M. Zaharna Blood Bank 2009

Duffy Blood Group

M. Zaharna Blood Bank 2009

Duffy Blood Group System

The Duffy blood group was discovered in 1950.

The Duffy glycoprotein is encoded by the FY gene,

The Duffy gene codes for a glycoprotein also found

The Duffy glycoprotein is a transmembrane protein

Five alleles at Duffy locus, the most important: Fya,

Fya is more immunogenic than Fyb

M. Zaharna Blood Bank 2009

M. Zaharna Blood Bank 2009

Fy(a-b-) blacks do not produce anti-Fya or

Fy(a-b-) Caucasians become sensitized

This suggest that Fy(a-b-) phenotype

M. Zaharna Blood Bank 2009

Fya, Fyb antigens are Destroyed by enzymes

Abs DO NOT agglutinate enzyme treated cells

Fya is more immunogenic than Fyb

M. Zaharna Blood Bank 2009

IgG antibodies and

Anti- Fyb is more