THE MNS (002) System

Background
• Landsteiner and Levine – discovered anti-M and anti-N in 1927
- M and N are antithetical
• Walsh and Montgomery – discovered S antigen (genetically linked to M and N)
- s antigen (antithetical of S) was discovered in 1951
• Family genetics (Molecular genetics) – there is a disequilibrium in the expression of S, s, M and N.
• Relative frequency in whites: Ns > Ms > MS > NS
Prevalence of Common MN and Ss Phenotypes

Phenotype Whites (%) Blacks (%)

M+N- 28 26
M+N+ 50 44
M-N+ 22 30
S+s- 11 3
S+s+ 44 28
S-s+ 45 69
S-s-U- 0 <1

U (“universal”) – named by Weiner

- All U- were also S-s-
M and N antigens
• Located at the outer end of Glycoprotein A (GYPA/GPA)
• Easily destroyed by enzymes ficin, papain and bromelin
• Heterogenous ( amino acids and carbohydrate chains)
S and s antigens
• Located farther down the glycoprotein B (GYPB/GPB)
• Less easily degraded by enzymes
ANTIBODIES
• ANTI-M and ANTI-N
 Cold reactive IgM or IgG (don not react at 37 degree Celsius)
 They do not bind complement and do not react with enzyme-treated RBCs
 Common in children and in patients with bacterial infections (anti-M)
 Seen in renal patients (anti-N)
 As long as anti-M does not react at 37 degree Celsius, it is not clinically significant
 Rarely causes HTR or HDFN
• ANTI-S and ANTI-s
 Mostly IgG and reactive at 37 degree Celsius
 Clinically significant
GPA- ad GPB- Deficient Phenotypes

1. U- Phenotype
2. En(a-) Phenotype 3. Mk Phenotype
• Located on GPB
• RBC type: S-s-U- • “envelop” • Named by Metaxas and
• Seen in RBCs of all • M-N- Metaxas-Buhler in 1964
individuals except 1% of • An allele did not produce
African americans who • No GPA is produced due
to rare gene deletion in M or N
lack GPB
• These individuals can the GPA locus • Mk gene is single, near-
make anti-U and reported • Can cause severe HTR complete deletion of both
to cause severe and fatal and HDFN GYPA and GYPB
HTR and HDFN
THE KELL (006) and Kx (019)
Systems
KELL Blood Group System
• It was the first blood group system discovered after the introduction of antiglobulin
testing
• Anti-K was identified in 1946 in the serum of Mrs. Kelleher
• Anti-k, antithetical partner of K, was described in 1949
• Kpa and Kpb were describe in 1957 and 1958 respectively
• Jsa (1958) and Jsb (1963)
• Kell antigens are not destroyed by enzyme papain and ficin but can be denatured by
trypsin and chymotrypsin when combined

*refer to Table 8-15

PREVALENCE OF COMMON KELL SYSTEM PHENOTYPES

PHENOTYPES WHITES (%) BLACKS (%)

K-k+ 91 96.5
K+k+ 8.8 3.5
K+k- 0.2 <0.1
Kp (a+b-) <0.1 0
Kp(a+b+) 2.3 Rare
Kp(a-b+) 97.7 100
Js(a+b-) 0 1
Js(a+b+) Rare 19
Js(a-b+) 100 80
ANTI-K

• Outside ABO and Rh antibodies, anti-K is the most common antibody

seen in the blood bank
• IgG, reactive to AHG phase
• Assoc. with HTR and HDFN
THE Kx Antigen
• Present on all RBCs except those of the rare Mcleod phenotype
• K0 and Kmod phenotypes have increased Kx antigen
• When Kell antigens are denatured, the expression of Kx increases.
The McLeod Phenotype and Syndrome

• McLeod, a student who appeared to be Kell null but demonstrated

weak expression of k, Kpb and Jsb
• Very rare, all who have it are males.
• Their RBCs are acanthocytic (having irregular shapes and protrusions)
with decreased deformability and reduced in vivo survival resulting to
chronic hemolytic anemia characterized by reticulocytsis,
bilirubinuria, splenomegaly, and reduced serum haptoglobin levels.
• Individuals with the McLeod phenotype have a variety of muscle and
nerve disorders, one of the neuroacanthocytosis syndromes.
THE DUFFY (008) SYSTEM
BACKGROUND
• Named after Mr. Duffy, a multiply transfused hemophiliac who in
1950 was found to have the first described example of anti-Fya
• Its antithetical antigen, Fyb, was found in the serum of a woman who
had three pregnancies
Fy (a-b-)
• Majority of African Americans with this null phenotype
• In 1975, it was observed that Fy(a-b-) RBCs resist infection in vitro by
the monkey malaria organism Plasmodium knowlesi and Plasmodium
vivax, human malaria
PREVALENCE OF COMMON DUFFY PHENOTYPES

PHENOTYPES WHITES (%) AFRICAN AMERICANS(%) CHINESE (%)

Fy(a+b-) 17 9 90.8

Fy(a+b+) 49 1 8.9

Fy(a-b+) 34 22 0.3

Fy(a-b-) Very Rare 68 0

THE KIDD (009) SYSTEM
BACKGROUND
• Consists only 3 antigens: Jka, Jkb, Jk3
• Found in serum of Mrs. Kidd, whose infant had HDFN
• Antibody: anti-Jka (John Kidd)
• Null phenotype: Jk(a-b-)
• Kidd antigens are not very immunogenic
PREVALENCE OF KIDD PHENOTYPES
PHENOTYPE WHITES(&) Blacks(%) ASIANS(%)

Jk(a+b-) 28 57 23

Jk(a+b+) 49 34 50

Jk(a-b+) 23 9 27

J(a-b-) Exceedingl rare Exceedingly rare 0.9 Polynesians

THE LUTHERAN (005) SYSTEM
BACKGROUND
• In 1945, anti-Lua was found in the serum of a patient with lupus
erythematosus following the transfusion of blood
• Anti-Lua was named after the donor, Lutheran (Lutteran but the
sample was mislabeled)
• Anti-Lub antithetical partner to Lua was described by Cutbush and
Chanarin
• Null phenotype: Lu(a-b)
PREVALENCE
PHENOTYPE MOST POPULATION (%)

Lu(a+b-) 0.15

Lu(a+b+) 7.5

Lu(a-b+) 92.35

Lu(a-b-) Very Rare

Lu(a-b-) Phenotypes
DOMINANT TYPE Lu(a-b-) RECESSIVE X-LINKED
• The expression of INHIBITOR TYPE
Lutheran was thought • Hemizygosity for a
to be duppressed by a mutation in the X-
rare dominant linked gene for the
regulator gene called major erythroid
In(Lu) for “Inhibitor og
Lutheran” transcription factor
GATA-I have been
• In(Lu) phenotype is
caused by inheritance
shown to result in
of a loss-of-function this X-linked
mutation inheritance
SUMMARY
• Clinically significant antibody specificities seen: M, P1, and I
• Antibodies to: K, S, s, Fya ,Fyb ,Jka , and Jkb are clinically significant