OTHER BLOOD GROUP SYSTEMS genotype substance red cell Le

phenotypes antibodies
Major Blood Group Systems ABH NONE ABH, Le(a-b-) Anti-Lea &
a. Lewis lele Anti-Leb
b. I sese
c. P ABH ABH ABH, Le(a-b-) Anti-Lea &
d. MNSs lele Anti-Leb
e. Kell SeSe/Sese
f. Kidd ABH Lea ABH, Le(a+b-) Anti-Leb
g. Duffy Lele/Lele
h. Lutheran sese
ABH ABH ABH, Le(a-b+) NONE
MAJOR BLOOD GROUPS SYSTEMS Lele/Lele Lea
Chromosome Locus SeSe/Sese Leb
Lea Se Leb
1 Duffy
4 MNSs Leb associated with receptor of H. pylori
7 Kell
18 Kidd Lewis system (Phenotypes and Frequencies)
19 Lewis, Lutheran Phenotype White (%) Black (%)
(a+b-)
Le 22 23
22 P1 (a-b+)
Le 72 55
(a-b-)
Le 6 222
Basic terms to remember Cord blood and red cells from newborn infants
Clinical significance: antibodies that is associated phenotypen as Le(a-b-)
with decreased RBC survival
Transfusion reactions Lewis Antigens
HDN Soluble antigens produced by tissues and found
Not clinically significant: antibodies that do not in body fluids (plasma)
cause red cell destruction Adsorbed on the RBC
Cold reacting antibodies: agglutination best
observed at or below room temp.

Warm reacting antibodies: agglutination best

observed at 37C

Systems that Produce Cold-Reacting Antibodies

Lewis Antibodies
A. LEWIS (007) SYSTEM
Lewis gene (Le) codes for production of
fucosyltransferase enzyme
Lewis system depends on Hh, Se, and Le genes
le, h, and se do not produce products
If the Le gene is inherited, Lea substance is
produced
Le, H and Se genes must ALL be inherited to
convert Lea to Leb
Usually occur naturally in those who are Le(a-b-)
Other phenotypes RARELY produce the
antibody
IgM (may fix complement, becoming
hemolytic)
Enzymes enhance activity
May be detected soon after pregnancy because
pregnant women may temporarily become Le(a-
b-)

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B. MNSs (002) SYSTEM Anti-M and anti-N antibodies
4 important antigens (more exist): Demonstrate dosage
M Anti-M and anti-N
N IgM (rarely IgG)
S Not clinically insignificant
s If IgG, could be implicated in HDN (RARE)
U (ALWAYS present when S & s are inherited; Will not react with enzyme treated cells
usually in Black population) Anti-Nf seen in renal patients, who are
M & N located on Glycophorin A dialyzed on equipment sterilized with
S & s and U located on Glycophorin B formaldehyde
Remember: Glycophorin is a protein that Anti-S, Anti-s Antibodies
carries many RBC antigens Clinically significant
IgG
MNSs System (Phenotype and Frequencies) Implicated with severe hemolytic transfusion
Phenotype Whites (%) Blacks (%) reaction with hemoglobinuria and HDN
M+N- 28 26 Anti-U Antibodies
M+N+ 50 44 Will react with S+ or s+ red cells
M-N+ 22 30 Usually occurs in S-s- cells
S+s- 11 3 Can only give U-negative blood units
S+s+ 44 28
Enhanced with enzyme treatment
S-s+ 45 69
S-s-U- 0 <1 MNSs Antibody Characteristics
M-N- : Resistant to P. Falciparum infection

MNSs Antigens

C. P (003) & PK (209) SYSTEM

Phenotype Detectable Ag Possible Ab

P1 P, P1 None
all show dosage P2 P Anti-P1
M & N give a stronger reaction when P (P null) None Anti-PP1Pk
homozygous, (M+N-) or (M-N+) (Anti-Tja)
Weaker reactions occur when in the P1k Pk, P1 Anti-P
heterozygous state (M+N+) P2k Pk Anti-P, Anti-
P1
Antigens are destroyed by enzymes (i.e. ficin,
papain)

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P and Pk (Phenotype and Frequencies) D. I (207) SYSTEM
Phenotype White (%) Black (%)
P1 79 94 Ii Antigens
P2 21 6 These antigens may be I or i
They form on the precursor chain of RBC
null
P (P ) Rare Rare
1
Pk Very rare Very rare Newborns have i antigen
P2k Most rare Most rare
Adults have I antigen
i antigen (linear) converts to I (branched) as the
P Antigen child matures (precursor chain is more linear at
Similar to the ABO system birth) at about 18 months
The most common phenotypes are P1 and P2
Rare i Adult (I negative) Phenotype
P1 consists of P1 and P antigens
P2 consists of only P antigens Individuals who do not change their I status
Like the A2 subgroup, P2 groups can produce after birth, usually in HEMPAS (Hereditary
anti-P1 Erythroblastic Multinuclearity with Positive
75% of adults have P1 Acidifies Serum) patients.
Strength of the antigen decreases upon storage
Anti-I antibodies
Found in secretions like plasma and hydatid
cyst fluid Anti-I
Cyst of a dog tapeworm IgM
Benign Anti-I = naturally occurring and
P Antibodies usually reacts only at 4oC
Anti-P1 Pathologic Anti-I = broader thermal range
Naturally occurring IgM of activity, reacting up to 30-32oC. Attach in
Not clinically significant vivo and cause autoagglutination and
Strong Anti-P1 was observed in individuals vascular occlusion (Raynauds
infected with hydatid disease (E. granulosus) phenomenon) or intravascular hemolysis
Associated with fascioliasis, C. sinensis and Autoanti-I = associated as a cause of Cold
O. viverinni infections agglutinin disease (similar to PCH). May be
Anti-P secondary to Mycoplasma pneumonia
Produced in individuals with paroxysmal infections
cold hemoglobinuria (PCH) Anti-i
Baphasic hemolysin: In PCH IgG auto- Mostly, IgM and reacts best with saline-
anti-P attaches complement when cold suspended cells at 4oC
(fingers, toes). As the red cells circulate, Rare and is sometimes associated with
they begin to lyse (releasing Hgb) Infectious mononucleosis (Epstein-Barr
This PCH antibody is also called the Donath- Virus)
Landsteiner antibody IgG anti-i has also been described and has
Anti-PP1-Pk ( originally called Anti-Tja) been associated with HDN.
IgM (sometimes IgG)
Reacts over a wide thermal range
Associated with spontaneous abortion in
early pregnancy

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Cold Antibodies (IgM) implicated in severe hemolytic transfusion
Anti-Lea reactions and HDN
Anti-Leb McLeod phenotype, affecting only males is
Anti-I described as a rare phenotype with decreased
Anti-P1 Kell system antigen expression
Anti-M The McLeod syndrome includes the clinical
Anti-A, -B, -H manifestation of abnormal RBC morphology
Anti-N and compensated hemolytic anemia and
LIiPMABHN neurologic and muscular abnormalities

Warm Antibodies (IgG) F. DUFFY BLOOD GROUP SYSTEM

Rh antibodies Fya and Fyb antigens are destroyed by enzymes
Kell and ZZAP; they are well developed at birth. The
Duffy Fy(a-b-) phenotype is prevalent in blacks but
Kidd virtually nonexistent in whites
S,s Fy(a-b-) RBCs were shown to resist infection
by the malaria organisms P. knowlesi and P.
Remember enzyme activity:
vivax
Anti-Fya and Anti-Fyb are usually IgG
antibodies and react optimally at the
antiglobulin phase of testing; both Antibodies
have been implicated in delayed hemolytic
transfusion reaction and HDN.

G. KIDD BLOOD GROUP SYSTEM

Remembering Dosage:
Kidds and Duffy the Monkey (Rh) eat lots of
M&Ns
E. KELL BLOOD GROUP SYSTEM
The blood group antigens are well developed at
birth and are not destroyed by enzymes
The kell blood group antigens are destroyed by
DTT, ZZAP and glycine acid-EDTA
Excluding ABO, the K antigen is rated second
only to D antigen in immunogenicity
The k antigen is high incidence antigen
Anti-K is usually an IgG antibody reactive in the
AHG phase and is made in response to
pregnancy or transfusion of RBC, it has been
Anti-Jka and Anti-Jkb demonstrate dosage, are
often weak, and are we found in combination
with other antibodies; both are typically IgG and
antiglobulin-reactive
Kidd system antibodies may bind complement
and are made in response to foreign RBC
exposure during pregnancy or transfusion
Kidd system antibodies are common cause of
delayed hemolytic transfusion reactions.
Kidd system antibody reactivity is enhanced
with enzymes, LISS, and PEG.
H. LUTHERAN BLOOD GROUP SYSTEM
Lua and Lub antigens produced by allelic
codominant genes; they are poorly developed at
birth
Anti-Lua may be naturally occurring saline
agglutin that reacts optimally at room
temperature.

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 Anti-Lub is an IgG antibody reactive at the
AHG phase; usually produced in response to
foreign RBC exposure during pregnancy or
transfusion
The Lu(a-b-) phenotype is race and may result
from three different genetic backgrounds

System Antigen Class Comments

Kell K, k, Kpa, IgG Antigens
Kpb, Jsa, in the Kell
Jsb system is
destroyed
by DTT
Duffy Fya, Fyb IgG Fy(a-b-) is
protective
against
malaria
Kidd Jka, Jkb IgG Associated
with
delayed
transfusion
reactions
Lutheran Lua, Lub IgM May
exhibit
mix-field
reaction

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