o Genes are written in italics or
LEC 3: BLOOD GROUP
TERMINOLOGY AND
OTHER BLOOD GROUP
SYSTEMS
INTRODUCTION
• The ABO and Rh Blood groups are
the most significant in transfusion
practice
• ABO being the most immunogenic.
• There are more than 300 RBC
antigens that are formally recognized
internationally.
BLOOD GROUP TERMINOLOGY
• Blood groups are determined by the
presence or absence of specific
antigens on the surface of red blood
cells.
• Most blood group alleles are
codominant and express a •
corresponding antigen.
o e.g., a person who inherits
alleles K and k expresses
both K and k antigens on his
or her RBCs.
• Some genes code for complex
structures that carry more than one
antigen
• Silent or amorphic alleles are genetic
variants that do not produce any
antigens on the surface of red blood
cells (RBCs). These alleles are rare
and result in what is known as a null
phenotype.
• Blood group antigens are detected by
alloantibodies, which can occur
naturally or as a response to exposure
to non-self red blood cell (RBC)
antigens through transfusion or
pregnancy.
• Blood group antigens have been
named using different styles of
symbols over the last century,
including uppercase single letters,
uppercase and lowercase letters for
alleles, letters derived from the
system's name (sometimes with
superscripts for alleles), letter
symbols followed by numbers, and
the more recent use of all uppercase
letters.
• Conventions are followed when
writing alleles, antigens, and
phenotypes.
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underlined, with the allele
number or letter as
superscript.
▪ e. g. (ISBT) LE,
MNS*1, MNS*2,
KEL*1, KEL*2
▪ (Conventional) Le,
le, M, N, S, Jka, Jkb
o Antigen names are written in
regular type without italics or
underlining, and some
antigens have numbers or
superscript letters.
▪ e.g. Lea, Leb, M, N,
S, Jka, Jkb
• A phenotype describes the presence
of antigens on an individual's RBCs
and is indicated by serologic test
results.
• Proper use of subscripts,
superscripts, and italics is important.
Phenotype representation depends
on antigen nomenclature and whether
letters or numbers are used.
• For letter antigens, a plus sign (+) or
minus sign (-) is used on the same line
to indicate presence or absence,
respectively.
o e.g. M+ and K–
• Antigens with superscripts use
parentheses to indicate the
superscript letter along with the
defining letter of the antigen.
o e.g. Fy(a+) and Jk(a–)
• When testing for both antithetical
antigens, both results are written
within parentheses.
o e.g. Fy(a–b+)
• Numerically designated antigens use
a colon after the defining letter(s) of
the system, followed by the number
representing the antigen.
• No plus sign is written for present
antigens, but a minus sign (-)
precedes negative results, and
multiple results are separated by
commas.
o e. g. Sc:–1,2
• Phenotypes of multiple blood group
systems are separated by semicolons
o e. g. S+s+; K–; Fy(a+b–)
• One must remember that serologic
tests determine only RBC phenotype,
not genotype.
 • Antibodies are described by their individuals with the Le(a-b-)
antigen notation with the prefix anti-, phenotype.
including a hyphen before the antigen • Lewis antibodies are frequently
symbol encountered in pregnant women.
• Lewis antibodies are not considered
For example: significant for transfusion medicine
o An antibody directed against
the A antigen would be The I and i Ags and Abs
described as anti-A. • The I and i antigens do not exhibit an
o An antibody specific to the antithetical relationship but rather
Rh(D) antigen would be have a reciprocal relationship.
denoted as anti-D. • Most adult RBCs are rich in I and have
o Similarly, an antibody only trace amounts of i antigen.
targeting the K antigen • Anti-I is typically a benign and weak
would be represented as antibody that occurs naturally in
anti-K. individuals
• Pathogenic anti-I antibodies, in
OTHER BLOOD GROUP SYSTEM contrast to benign anti-I antibodies,
can exhibit stronger reactivity and
The Lewis (007) System pose a higher risk of complications.
• The Lewis blood group antigens are • In patients with Mycoplasma
not synthesized by the red blood cells pneumoniae infections, it is possible
(RBCs) themselves. for them to develop strong cold
• Instead, these antigens are adsorbed agglutinins with autoanti-I specificity.
or acquired from plasma onto the RBC • Anti-i is an infrequent IgM agglutinin
membrane. that demonstrates its highest
• The Le gene encodes for L- reactivity at a temperature of 4°C.
fucosyltransferase enzyme, which Particularly strong instances of anti-i
adds L-fucose to type 1 chains. antibodies have been linked to cases
• The Le gene is required for the of infectious mononucleosis.
expression of Lea substance.
• Both Le and Se genes are necessary The P Blood Group
for the formation of Leb substance. • Similar to the ABO System
• The Le(a+b+) phenotype is • The most common phenotypes are
associated with the presence of Lea P1 and P2
and Leb antigens. o P1 – consists of P1 and P
• The lele genotype is more prevalent in antigens
individuals of African descent o P2 – Consists of only P
compared to individuals of European antigens
descent. • P Antigens:
• The lele genotype results in the Le(a- o Decreases upon storage
b-) phenotype, indicating the absence o Poorly developed at birth
of both Lea and Leb antigens. • P Antibodies
• The Le(a-b-) phenotype is commonly o Anti-P1
found among individuals of African - Naturally occurring
ancestry. IgM
• The absence of Lea and Leb antigens - Not Clinically
in the Le(a-b-) phenotype is due to the Significant
lack of functional L-fucosyltransferase o Anti-P
enzyme encoded by the lele - an be of the IgM or
genotype. IgG class and are
• Lewis antigens are poorly expressed typically produced
on red blood cells (RBCs) at birth. in response to
• Lewis antibodies, which are generally exposure to the P
IgM antibodies, are naturally antigen through
occurring and primarily produced by

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 blood transfusion or ➢ Kell antigens have disulfide-bonded
pregnancy. regions on the glycoproteins making them
sensitive to sulfhydryl regaents:
The MNS Blood Group ▪ 2- mercaptoethanol (2-ME)
▪ Dithiothreitol (DTT)
4 important antigens: ▪ 2-aminoethylisothiouronium
o M bromide (AET)
o N
o S Other Kell anitgens
o S ➢ Kp antigens
o U (ALWAYS present when S - Kpa is a low frequency antigen
& s are inherited) (only 2%)
- Kpb is a high frequency antigen
➢ M & N Antigens – primary antigens; (99.9%)
located in Glycophorin A ➢ Js antigens
➢ S & s Antigens – located in - Jsa (20% in blacks, 0.1% in
Glycophorin B whites)
➢ MNSs antigens all show dosage - Jsb is high frequency (80-100%)
• Single Dose / ➢ Kellnull or K0
Heterozygous: M+N+ - rare phenotype in the Kell blood
(Weaker reaction) group system where an individual
• Double Dose / lacks the expression of Kell
Homozygous: M+N- antigens on their red blood cells
(Stronger reaction) (RBCs).
- As a result of transfusion, Ko
➢ Antigens can be destroyed by individuals can develop anti-Ku
enzymes - Rare Kell negative units should be
➢ Anti – M and Anti – N: demonstrate given
dosage
- these antibodies are typically Kell Antibodies
of the IgM class ➢ typically, of the IgG class
- they can cause agglutination ➢ Produced as a result of immune
(clumping) and hemolysis stimulation
(destruction) of RBCs if ➢ Anti-K is the most common because
incompatible blood is the K antigen is extremely
transfused. immunogenetic
➢ Anti - S and Anti- s ➢ k, Kpb an Jsb antibodies are rare
- These antibodies are usually (many individuals who have these
of the IgG class antigens and won't develop
- can cause hemolytic antibodies)
transfusion reactions and McLeod Phenotype
hemolytic disease of the ➢ a rare blood group phenotype that is
newborn (HDN) associated with a specific genetic
➢ Transfusion Reactions and HDN: condition called McLeod syndrome.
Incompatible transfusions ➢ characterized by the absence or
reduced expression of the Kell blood
The Kell Blood Group group antigens on red blood cells
(RBCs)
Important antigen: ➢ caused by mutations or deletions in
o K (Kell) < 9% of the population the XK gene located on the X
o K (cellano) >90% of the population chromosome.
McLeod Syndrome
➢ Well developed at birth and not destroyed ➢ X-linked disorder
by enzymes ➢ caused by mutations or deletions in
➢ The K antigen is very immunogenic (2nd to the XK gene, which is located on the
the D antigen) in stimulating antibody X chromosome
production

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 ➢ Causes neurological symptoms, The Lutheran Blood Group
blood abnormalities (acanthocytosis, • 2 codominant alleles:
reticulocytosis), Muscle problem and ➢ Lua
cardiac and respiratory involvement ➢ Lub
• The antigens associated with the
The Kidd Blood Group Lutheran system show weak
Antigens expression on cold blood cells.
➢ Consists of 2 major antigens: • The Lub antigen, found in 92% of
(codominance) individuals, results in the Lu(a-b+)
o Jka phenotype
o Jkb • The Lu(a-b-) phenotype, lacking the
➢ Show dosage Lub antigen, is considered rare.
➢ Well developed at birth
➢ Enhanced by Enzymes Anti-Lua
➢ Not very accessible on the RBC ➢ IgM and IgG
membrane ➢ Not clinically significant
Antibodies ➢ Reacts at RT
➢ Anti-Jka and Anti-Jkb ➢ Causes mild HDN
➢ IgG ➢ Naturally occurring or immune
➢ Clinically significant stimulated
➢ Common Cause of delayed HTR Anti – Lub
➢ Usually appears woth other ➢ Rare because Lub is high incidence
antibodies when detected antigen
➢ IgG
The Duffy Blood Group ➢ Associated with transfusion recations
Antigens (rare HDN)
➢ Predominant genes (Codominant
alleles): The HTLA Antigens
o Fya and Fyb code for • High-Incidence Antigens on the
antigens that are well Lymphocytes and Platelets.
developed at birth • HLA-A1, HLA-A3, HLA-B8, and HLA-
➢ Antigens are destroyed by enzymes B12
➢ Show dosage • Important in the field of transfusion
Antibodies medicine and organ transplantation
➢ IgG
➢ Do not bind complement
➢ Clinically significant
➢ Stimulated by transfusion or
pregnancy (but not a common cause
of HDN)
➢ Do not react with enzyme treated cells

Duffy and Malaria

• Individuals with the Fy(a–b–) blood
group phenotype have RBCs that are
resistant to infection by the malaria
parasite Plasmodium vivax.
• The Duffy antigens serve as receptors
for the P. vivax parasite, allowing it to
invade and infect red blood cells.
• This natural resistance to P. vivax
malaria is more commonly observed
in populations of African descent, as
the Fy(a–b–) phenotype is more
prevalent in individuals of African
ancestry.

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