Blood Group Terminology and the Other Blood Groups

Major and Minor Blood Groups

Chromosome 1 Rh
Major Blood Group Minor Blood Group Systems: Duffy
-Diego Scianna
Systems other than ABO
-Cartwright Cromer
and Rh:
-Xg Knops
-Kell Chromosome 2 Gerbich
-Scianna
-Duffy -Dombrock Chromosome 4 MNS
-Kidd -Colton Chromosome 6 Chido/Rodgers
-MNSs -Chido-Rodgers Chromosome 7 Cartwright
-P -Gerbich Colton
-I -Cromer Kell
-Knops Chromosome 9 ABO
-Indian Chromosome 11 Indian
-JMH Chromosome 12 Dombrock
Chromosome 15 JMH
Chromosome 17 Diego
Chromosome 18 Kidd
Chromosome 19 H
Lewis
Blood Group No. of Ags Landsteiner-Weiner
Lutheran
Chromosome 22 P
Chromosome X Xg
Kx
 MNS System (002)

Symbol Gene Antithetical -No. of RBC Antigen Expression at Birth Antigen Antibodies Demonstrates Dosage Clinical Significance
Discover Found in Antigens Enzymes Distribution: Complement Binding Antigen:
er -Antigen
Plasma/RBC -HDFN
-Incompatible Crossmatch
MNS  GYPA and GYPB, GYPE gene MN 46 -Well developed at birth. -Plasma
codominant Ss (M&N, S&s) Anti-M: Anti-M Anti-N Anti-S & Anti-s Anti-M Yes
Antibody:
considered as ancestral gene MN -(some)pH -React better with M+N-
Enzyme M& S&s - IgG RBCs (MM) Anti-M -Common in bacterial
-IgG -IgM or IgG
Landst Chromosome 4 White Ss N dependent: Ab reactivity enhanced infection px.
-Naturally -saline - reactive at
einer M and N Ag frequency: U Ficin   pH 6.5 37°C by: -more common in
occurring Agglutinin
and →Glycophorin A (GPA) Ns>Ms>MS Papain   -(some) react saline -cold- & antiglobulin serum to cell ratio children.
>NS on RBCs  or  Incubation
Levine S and s Ag Bromelin   agglutinins reactive. test. -Rarely cause HTR,
time
→Glycophorin B (GPB) Trypsin  X exposed to that react - few express  Incubation decreased red cell survival
  Glucose <30°C. optimal temperature or HDFN.
Pronase
solutions. -Do not bind -Do not bind reactivity Adding potentiating As long as anti-M
Pos M N ZZAP: 
complement complemen between 10°C medium: doesn’t react at 37°C, it is
itio -DTT + (Papain X
-Do not t and 22°C by -Albumin not clinically significant.
n + fucin)
react with -Do not saline indirect -Low ionic strength saline
-2-
solution(LISS)
1 Serine Leucine aminoehtylisoth X enzyme react with antiglobulin Anti-N -not clinically significant
iouronium -polyethylene glycol
treated enzyme test. unless it reacts at 37°C.
5 Glycine Glutam bromide (PEG)
-Chloroquine RBCs. treated - may - rare cases
ic Acid -Glycine-acid RBCs. bind of mild HDFN.
Anti-N Yes
EDTA treatment X complement
Pos S s - React better with M–N+ - seen in renal patients
X
itio (NN) RBCs. who were dialyzed on
n equipment sterilized with
Anti-S -Yes
and Anti-s -may or may not react formaldehyde.( anti-Nf)
29 Methio Threoni Anti-S and -severe HTRs with
with enzyme-treated
nine ne Anti-s hemoglobinuria.
RBCs, depending on
the extent of - HDFN
Amount in degradation
treatment and the  Units selected for
depends on: transfusion must be
GPA- and GPB –deficient efficiency of the
Strenght of enzyme enzyme. antigen-negative and
Phenotypes: crossmatch-compatible.
solution U-Phenotype
Lenght of treatment En(a-) Phenotype
Enzyme to enzyme ratio. Mk Phenotype
a
U and En Autoantibodies
GPA and GPB are
GPAM
expressed on renal
endothelium and
epithelium.
 The P Blood Group: P1PK (003) and Globoside (028) Systems and Related (209) Antigens

Symbol Gene Antithetical No. of Antigens RBC Antigen Antigen Antibodies Demon Clinical Significance
Discover Found in Antigen Expression at Birth Distribution: Complement Binding strates
er Enzymes Plasma/RBC Dosage
P1PK -P1PK gene -Inherited 2 P1 Antigen P blood group exist as No
(chromosome independently. poorly expressed at glycosphingolipids. Anti-P1 k Alloanti-P Anti-P1 k Alloanti-P
Anti-PP1 Anti-PP1
Landst 22) birth P blood group
P- P1PK blood group -IgM -IgM and IgG -IgG -Rare anti -P1 react -severe HTRs and -associated with
-P gene may take up antigens are
einer system -Naturally -Originally called -naturally at 37°C causes in HDFN. habitual early
(Chromosome 3 to 7 years to be fully synthesized by a
and P1 - Globoside occurring anti-Tj occurring vivo RBC -increased abortion.
-B3GALNT1 expressed. sequential action of
Levine Pk –Globoside -Weak cold -(the T in the Tja alloantibody destruction. incidence of
gene encoding glycosyltransferase. refers to tumor)
the 3-β-N- collection Resistant to Precursor P1 can be reactive saline in the sera of -Both immediate spontaneous
-react over a wide
acetylgalactosa  Luke-Globoside treatment with: glycosylated to type agglutinin Pk individuals. and delayed HTRs abortions in early
thermal range and
minyltransferase collection ficin 2H chains (carry ABH -Reactive at 4°C. efficiently bind -potent have been reported. pregnancy
(Gb4 synthase) The discovery of papain chains). -Bind complement, hemolysin -parasitic infections -early abortions
responsible for strong anti-P1 in two Two common DTT common precursor is complement which makes reacting with are associated with with anti-PP1Pk
converting Pk to P1 individuals phenotypes: chloroquine lactosylceramide (or -detected in them potent all cells except anti-P1. or anti-P
infected with antiglobulin test hemolysins. the -Pk antigen is a
P. -P1 glycine-acid Gb2, also known as -Recent studies
Echinococcus when -produced by p autocontrol receptor for shiga
-Gene encoding -P2 EDTA. ceramide dihexose or demonstrate
granulosus polyspecific individuals early and those toxins, which cause
the enzyme Three rare CDH). k
that P provides
tapeworms led to the (anti-IgG plus in life without with the p shigella dysentery
responsible for phenotypes: Blacks have a P1, P, or Pk found
identification of P1 anti-C3) RBC phenotype. and E. coli some protection
the synthesis -p stronger expression on:
and Pk substance in reagents are sensitization -individual associated against HIV
of Pk, a 4-α- -P1k of P1 than whites. RBCs
hydatid cyst fluid. used. and reacts with making the hemolytic uremic infection of
galactosyltransf -P2k Rare dominant lymphocytes
Strong antibodies The P1 all RBCs except antibody may syndrome. peripheral blood
erase (Gb3 or Pk gene for the In(lu) granulocytes
to P1 have also been antigen those of the type P1+ mononuclear
synthase) type Lu(a–b–) RBCs monocytes
found in patients with
Phenotype React deteriorates p phenotype. cells.
with inhibits the P found on:
fascioliasis (bovine expression of P1 so platelets rapidly on Autoanti-P
Anti-P:anti-P1 P1 Anti-P1
liver fluke disease) that P1 individuals epithelial cells storage. -cold-reactive IgG autoantibody in patients with paroxysmal
Anti-P
P+:P1 and in bird handlers. Anti-P who inherit this fibroblasts. cold hemoglobinuria (PCH).
P2
P–:P2 modifier gene may P and Pk Luke(LKE) Antigen -Historically, this rare autoimmune disorder was seen in
P Anti-P
P null:p type serologically as found in: patients with tertiary syphilis; now commonly presents as a
P1k Anti-P1 -Tippett andc olleagues
P1–. plasma as transient, acute condition secondary to viral infection,
Anti-P
k -antibody in the serum of a patient with Hodgkin’s
(glycosphingolipids) especially in young children.
lymphoma that divided the population into three
P2k Anti-P
k
-biphasic hemolysin
as glycoproteins in phenotypes:
p No rx: -In vitro, the antibody binds to RBCs in the cold, and via
Anti-P1
hydatid cyst fluid. -84% tested Luke+
complement activation, the coated RBCs lyse as warmed to
Anti-P -14% were weakly positive or Luke(w) 37°C.
k
Anti-P -2% were Luke– -demonstrable only by the Donath-Landsteiner test.
P system antigens serve as receptors for P-fimbriated
Pnull are more uropathogenic E. coli—a cause of urinary tract infections.
common in Japan, P is the receptor of human parvovirus B19.
North Sweden and
in an Amish group
in Ohio.
 The Lutheran (005) System

Symbol Gene Antithe No. of Antigens RBC Antigen Antigen Antibodies Demonst Clinical Significance
Discoverer Antigen Expression at Birth rates
Found in tical Distribution: Complement Binding
Enzymes Dosage
Plasma/RBC
LU -LU gene Yes 20  poorly developed Lutheran Anti-Lu
a b
Anti-Lu Yes HDFN is rare and only mild.
-chromosome 19 Anti-Lua  Lua and Lub at birth glycoprotein is widely
-IgM -IgM and IgA Anti-Lua Anti-Lub
-Cutbush and -located on type 1 Anti-Lub Lu(a–b–) Phenotypes detected on fetal distributed in tissues:
- naturally occurring -IgG; reactive at 37°C -encountered as an -made in response
Chanarin transmembrane -brain incompatible to
Dominant Type Lu -Inheritance of a loss-of- RBCs as early as 10 to saline agglutinins at the antiglobulin
a protein -lung crossmatch or pregnancy or
-anti-Lu was (a–b–) function mutation on one 12 weeks of gestation that react better at phase
- codominant -pancreas during an antibody transfusion.
found in the allele of EKLF. room temperature
genes -placenta workup for another -implicated with
serum of a -carry trace amounts of Lutheran
than at
-Lu(a–b–) Enzyme -skeletal muscle specificity. shortened survival
patient with Lutheran antigens in Ag 37°C. -anti-Lua that react of transfused cells
phenotype -hepatocytes (fetal
lupus adsorption-elution test. Ficin X - A few react at 37°C only at and post
inherited as a hepatic epithelial
erythematosu -dominant type Lu(a–b–) RBCs Papain X by indirect temperatures <37°C transfusion
recessive silent cells).
s, following have reduced expression of trypsin  antiglobulin test. are jaundice.
allele CD44 and a weak expression of The presence of clinically -severe or acute
transfusion of -  - Some are capable of
- Lutheran antigen b Lutheran glycoprotein insignificant. hemolysis has not
a unit of P1, i, AnWj, MER2, and In chymot binding complement.
expression is blood group antigens. on placental tissue -only rare and mild been reported.
blood rypsin characteristic loose,
variable from one Recessive Type Lu -recessive inheritance may result in delayed HTRs.
carrying the DTT No rx mixed-field reactivity
individual (a–b–) -two rare silent adsorption of
correspondin AET No rx in a test tube.
to another. alleles LuLu at the Lutheran maternal antibodies
g low-
Lutheran locus. to Lutheran antigens
prevalence Anti-Lu3
proteins are -lack all Lutheran antigens
antigen. rare antibody that reacts with all RBCs
multifunctional -make an inseparable anti- Alloanti-Lub
-made anti-c, except Lu(a–b–) RBCs.
adhesion Luab called Reacts with all cells tested except the
anti-N, the
molecules that anti-Lu3 The antibody looks like inseparable autocontrol, and reactions are often
first example
of anti-CW,
bind laminin, Recessive X-Linked -large Australian family did anti-Luab and recognizes a common weaker with Lu(a+b+) RBCs and cord
notably in sickle RBCs.
and anti- Inhibitor Type not fit either the dominant antigen
cell disease
Levay, now or recessive inheritance Lu3, that is present whenever Lua or Lub
c patterns.
known as Kp is present (much like the Jk3 association
-Lu(a–b–) family members
with Jka and Jkb)
were male and carried trace
amounts of Lub detected by usually antiglobulin reactive
adsorption-elution. made only by individuals with the
-The pattern of inheritance recessive type of Lu(a–b–).
suggested an X-borne
inhibitor to Lutheran.
-Locus XS, XS1- common
allele; XS2 the rare inhibitor
that suppresses in a
hemizygous state.
 The Kell (006) and Kx (019) Systems
Symbol Gene Antith No. of Antigens RBC Antigen Antigen Antibodies Demons Clinical Significance
Discoverer Found in etical Antigen Expression at Birth Distribution: Complement Binding trates
Enzymes Plasma/RBC Dosage

KEL KEL gene Yes 32 Detected on fetal RBCs -found only Occasion
K and k antigens as early as 10 weeks. on RBCs. ally
Anti-K -IgG The McLeod Phenotype and
-serum of Mrs -Chromosome 7 K-k a b c Well developed at -the -Reactive in antiglobulin phase; agglutinate Syndrome
Kelleher Kell antigens are Kp , Kp and Kp Antigens associated Xk
Kpa birth. saline-suspended RBCs.  McLeod
-first blood located on a a b protein is - usually made in response to antigen -Absence of Kx proteins in
Kpb Js and Js Antigens Total number of K
group system glycoprotein-731 found in exposure through pregnancy RBCs membrane lead to
discovered amino acids Jsa K -rated second only on D in antigen sites per RBC: erythroid and transfusion. McLeod Phenotype
after Kx protein (440 JSb immunogenicity. -only 3,500 to 18,000 tissues such -Most common antibody seen in blood bank. -initially appeared to be Kell
introduction amino acids) -9% prevalence in whites. sites as: -IgM (bacterial infections) null but demonstrated weak
of -Kell glycoprotein is k seldom encountered brain
K is very -Some react poorly in methods incorporating expression of k, Kpb and Jsb
antigloobulin covalently linked by a -2% of whites. immunogenic despite lymphoid LISS and some automated system. IAT is the detectable by adsorption-
testing. a single disulfide Kp organs
-associated with suppression of other Kell its lower quantity. most reliable method of detection. PEG may elution methods.
bond. antigens in the same molecule, including k heart reactivity. very rare.
b skeletal
Kp and Jsb. -implicated in severe HTRs and HDFN (fetal X-linked inheritance(males)
XK Gene encodes Kx Enzyme Kell muscle anemia).
(Kpa & Kpc) Low prevalence mutations of Acanthocytic RBC w/
antigen, which is a Ag Binds complement (extravascular)
their high prevalence partner Kpb deformability and in vivo
precursor of of Kell Ficin X -Kell -stillbirth (<64 titer)
The effect appears to result from a survival.
Ags Papain X glycoprotein -If father is K+, mother is K-, the fetus should
reduced amount of the Kell Chronic Granulomatous
-Kel genes convert is expressed be monitored carefully for signs of HDFN.
a Trypsin +  Disease
Kx substance into glycoprotein(produced by Kp on fetal RBCs a a
the Kell Ags on Chymotrypsin at a much Anti-Kp -Naturally occurring(Anti-Kp ) Absence of Kx proteins in
c -rare
RBCs Kp Thiol-reducing earlier stage a -rare WBCs cause CGD (Theory before
Anti- Js and
-short arm of the X agents: of -most often detected through unexpected that is not valid recently)
a -20% of blacks (prevalence is 10X greater other Low-
chromosome. Js 100 to 200 erythropoiesis incompatible crossmatches or cases of HDFN. Leukocytes are able to
than K antigen.)  Prevalence
b mM DTT than Rh -most antibodies result from transfusion or
Js -0.1% of whites Kell Ags phagocytose (due to inability to
2- antigens. pregnancy.
-These ags were linked to the Kell system mercaptoetha make NADH-oxidase) but not to
K (Kell) & k (cellano) 
are produced by when it was discovered that K0 RBCs were nol (2-ME) Anti-k -rare kill bacteria

allelic genes, this Js(a-b-). AET  b -easy to detect but difficult to work with. Patients with CGD have
ZZAP Anti-Kp
results into 3 -Testing an unidentified high-prevalence recurrent bacterial infections
Glycine-acid  b
phenotypes: Kx -Present on all RBCs except those of rare Anti-Js and antibody against DTT- or AET- treated RBC is Patients who lack Kx on RBCs &
 K+k- (genotype Antigen McLeod phenotype. EDTA other High helpful technique. WBCs have both Mcleod and
KK) -Ko and Kmod Phenotype RBCs have Kx Prevalence -Finding compatible units for transfusion can CGD
K+k+ (genotype Kk) antigen. Kell Ags be difficult; siblings and rare-donor
Kell Null (K0) Phenotype
K-k+ (genotype kk) -Lack expression of all Kell antigens. inventories are the most likely sources.
Ko K0 is a silent Kell allele
Other allelic genes -No membrane abnormality and survive Anti- Ku
Phenoty -Made by immunized individuals with K0 When homozygous K0K0
include: normally in circulation. (K5)
a b pe phenotype. inherited no Kell system
Kp / Kp -rare phenotype
-Recognizes the “universal” Kell antigen (Ku)
antigens are expressed.
 a
Js / Jsb present on all RBCs except Ko Kx antigen expression is
enhanced
-caused both HDFN and HTRs.
Very rare
 anti- Ku (Universal Kell)
The Lewis (007) System
Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression Antigen Antibodies Demon Clinical
Discoverer Found in -Antigen at Birth Distribution: Complement Binding strates Significance
Enzymes Plasma/RBC Dosage
LE LE gene No 6 Lewis antigens are not found in saliva as No
chromosome 19: result from the interaction of two Le a expressed on cord RBCs glycoproteins. Anti-Le a
Anti-Le b
Anti- Anti
named after one of Lewis gene (FUT3) is fucosyltransferases encoded by during pregnancy. not intrinsic to RBCs a b
 Le b most  Usually IgM agglutinin Le -Le
the first individuals but are on type 1
linked to Se (FUT2) and independent genes, Le and Se Lewis glycolipids are commonly Does not fix complement as Rare
to make the Lec glycosphingolipids hemoly
H (FUT1) where all not detectable in the encountered of readily as Anti – Lea.
antibody, (glycolipids) that are
located. Le(a+b-) ABH Non-Secretor Led plasma until about 10 the Lewis tic
reported by passively adsorbed to infrequently made by transfu
-Lewis gene (Le, le) -Lea substance is secreted Lex (X)- type 2 isomer of Lea days after birth. True antibodies and
Mourant in 1946. the RBC membrane Le(a+b–) individuals and sion
is often reactio
-Secretor gene (Se, se) regardless of the secretor Ley (Y)- type 2 isomer of Leb Lewis phenotype, after from the plasma. classified into 2 categories
detected in ns
 Only system Le Inheritance status. about 6 years. -Lewis enzyme has
Other Lewis Antigens room Anti- -Reacts best (HTR)
not ge codes for the -All Le (a+b-) individuals are found on lymphocytes been detected in
manufactured nonsecretors of ABH Leab ALeb and BLeb saliva, milk, temperature LebH when both -Associated
ne addition of and platelets and other tests Leb and H
by the red another L- substances. submaxillary glands, with factors
present on all result from tissues such as: Often of IgM antigens are
blood cell fucose that is Le(a-b+) ABH Secretors gastric mucosa, and causing certain
Le(a+b–) and the addition -pancreas class however, present on
(RBC) added to the -Secrete Lea and Leb kidney and cyst fluids diseases, such
Le(a–b+) of the A or B some may have the RBC such
 Manufactured subterminal
-
A result of genetic -stomach and has not been as peptic ulcers,
RBCs and on immunodomi IgG as group O
by tissue cells N- interaction of Lele and Sese -intestine detected in plasma or
components or and A2 cells. ischemic heart
and secreted acetylglucosa genes. 90% of cord nant sugar, to -skeletal muscle in RBC stroma.
RBCs. The type 1H chain may be entirely -represents an disease, cancer,
into body mine and thus -Some precursor chains are  gastrointestinal tract
-renal cortex IgG. antibody to a and kidney
fluids b not acted on by the Se antigen was in individuals is thought to be the
forms Le previously who have at -adrenal glands compound transplant
 Referred to as fucosyltransferase but may primary source of
antigen. known as Lex least one Se Enzyme Lewis antigen.
a system rather accept L-fucose from the Lewis glycolipid in rejection.
Se inheritance -fairly and one Le Ags Anti- Recognizes
than a blood Lewis fucosyltransferase plasma. -Lack of
group because ge codes for the
forming Lea common and allele. Ficin X -are readily shed from LebL any Leb
ne enzyme -2- antigen expression of
they are found -
Both Lea soluble and Leb is frequently Se converts Papain X transfused RBCs
fucosyltransfe found with type 1 chains Dithiothr X regardless of Lewis ags has
primarily in the soluble antigens can be within a few days of
rase, which anti-Lea or to type 1H, eitol the ABO type. been
secretions and found in the secretions but transfusion. -Lewis
adds L-fucose anti-Leb. providing a (DTT) IgM demonstrated
the plasma only Leb soluble antigens blood group
 Depends not to type 1 -The antibody suitable Glycine X substance present Often naturally occurring and made by Le (a- on the RBCs of
adsorbs onto the RBC from
only on the precursor is acceptor for acid in transfused plasma b-) persons.
plasma. patients with
inheritance of substance and heterogenous the A and B EDTA neutralizes Lewis binds complement.
thus forms -Leb has receptors for cancer,alcoholic
Lewis genes and occurs carbohydrates. antibodies in the cause in vivo hemolysis of red cells
type 1H . Helicobacter pylori cirrhosis, & viral
but also on the mainly in recipient. Sometimes reacts at 37◦C and Coombs phase
 must be Le(a-b-) Secretors or non- more weakly than at room temperature and parasitic
inheritance of Le(a–b–) -Also detected on the
present for a secretos Enhanced by enzymes infections due
the secretor secretors of surface of platelets
precursor -found frequently in Occur quite frequently in the sera of pregnant
gene group A1, B, and lymphocytes, to abnormal
substance to Africans. women
or A1B. The regardless of the lipid
be converted Transformation of Lewis phenotype after Rarely cause in vitro hemolysis
antigens now secretor status, but metabolism,
to Lea; birth seen in individual who inherit Le and known as Lex Anti – Lex -Agglutinates all Le (a+b-) and Le
not on the surface of changes in
Se gene Se genes:
 must be Le (a-b-) to Le (a+b-) to Le (a+b+) to Le (a- and Ley are Reactivity of Lewis granulocytes or (a-b+) RBCs triglycerides
present for b+) (the true phenotype) products of antibodies can be monocytes. -Formed by all individuals who and HDL,
conversion to FUT3 on type greatly enhanced by are phenotyped as Le (a-b-) and/or other
Leb. 2 precursor testing with enzyme- neoplastic
chains. treated RBCs;
changes
Hemolysis of enzyme-
treated RBCs may be occurring in
seen if serum is cancer patients.
tested.
The Duffy (008) System

Symbol Gene Antitheti -No. of Antigens RBC Antigen Expression Antigen Antibodies Demonstrates Clinical Significance
Discoverer Found in cal -Antigen at Birth Distribution: Complement Binding Dosage
Enzymes Plasma/RBC
FY FY (DARC) gene Yes 5 identified on fetal Yes
Fya- Fyb Fya, Fyb RBCs as early as 6 identified in: Anti-Fy a
Anti-Fy b reacting more Anti-Fya Anti-Fyb
-was named for Mr.  Fya Fyb weeks gestational age Brain strongly with RBCs
Chromosome 1 -common antibody -20 times less
Duffy, a encode colon that have a double associated with acute
FyFy appeared to be a 13,000 to 14,000 Fya or Fyb sites on Fy(a+b–) and Fy(a– well developed at -occurs three common than
multiply transfused the endotheliu dose than RBCs from and delayed HTRs
b+) RBCs, half that number of Fya sites on Fy(a+b+) RBCs. birth. times less anti-Fya and often
common genotype in antithetic m heterozygotes
hemophiliac who in frequently than occurs in
blacks, especially in al Ezymes FY lung some reagent RBCs
1950 was found to anti-K. combination
Africa; the gene is antigens. Fya Most important Duffy antigens in routine Ags spleen that appear to be
have the first  with other
Fy b blood bank. Ficin  thyroid from homozygotes
described example exceedingly rare in  antibodies.
three Papain thymus (and have a double
of anti-Fya. whites. Fy -silent allele  -IgG
-Fyb, was found in common Bromelin kidney cells dose of either Fya or
Fya glycine -major allele in blacks  -react best at the antiglobulin phase.
the serum of a alleles at Chymotrypsi Fyb) may actually be
Fyx - described in 1965 as a new allele at the Fy -bind complement.
woman who had the Fy n from heterozygotes if
Fyb aspartic locus  -do not react with enzyme-treated RBCs,
had three locus: ZZAP: they are from black
aci - inherited weak form of Fyb that reacts with this is a helpful technique when multiple
pregnancies Fya Papain/ficin donors; a silent allele,
Duffy glycoprotein is some examples antibodies are present.
Fy(a–b–) Fyb + DTT Fy, is commonly
a member of the of anti-Fyb. Rare autoantibodies with mimicking Fya
RBCs resist Fy DTT X found in blacks.
superfamily of - have depressed expression and Fyb specificity
infection in vitro by AET X
chemokine receptors of their Fy3 and Fy5 antigens. Anti- Fy3 Anti- Fy5
the monkey malaria Glycine-acid X
known as the Duffy -no anti-Fyx. -rare antibody - reported in
organism EDTA
Fy3 -found in the serum of an Fy(a–b–) white  made by Fy(a–b–) multiply
Plasmodium antigen receptor for Neuraminad
Australian female. It reacted with all RBCs mol. individuals who transfused Fy(a–
knowlesi. chemokines (DARC). ase
tested except those of the Fy(a–b–) Wgt. lack the Duffy b–) sickle cell
- resist infection by being a receptor for phenotype. Purified X glycoprotein. patients with a
P. vivax (one of the the malaria parasite P. -inseparable anti-FyaFyb, it was thought to Trypsin mixture of other
organisms causing
vivax, the Duffy react with an antigenic determinant or antibodies.
malaria in humans).
glycoprotein binds a precursor common to both Fya and Fyb
This discovery
variety of -not destroyed by enzymes.
provides an
Fy5 - reacted with the cells from an Fy(a–b–)Fy:–3
explanation for proinflammatory
white female, but it did not react with Fy(a+)
the predominance cytokines. or Fy(b+) Rhnull RBCs and reacted only weakly
of the Fy(a–b–)
with Fy(a+) or Fy(b+) D- RBCs.
phenotype in
- result of interaction between the Rh
persons originating The Fy(a–b–) complex and the Duffy glycoprotein.
from West Africa. phenotype has been - not destroyed by enzymes.
found in whites, Cree
Indian families, and
blacks

The Kidd (009) System

Symbol  Gene Antithetical -No. of Antigens RBC Antigen Expression Antigen Antibodies Demonstrates Dosage Clinical Significance
Discoverer Found in -Antigen at Birth Distribution: Complement Binding
Enzymes Plasma/RBC
JK JK (SLC14A1) Yes 3 well developed on the RBC only Yes common cause of HTR
gene Jka-Jkb Jka -commonly RBCs of neonates Anti-Jka b
Anti-Jk
 Allen & found on  often weak
Jka detected on -more frequently
colleagues Chromosome RBCs of most fetal RBCs as  found in combination with
Phenotype encountered than
reported 18 individuals. early as other antibodies, all of which
anti- Anti-Jkb, but
finding an Jk(a+b-) -91% of 11weeks make them difficult to detect.
neither antibody
antibody in The Jk(a+b+) blacks and b detected at 7 react more strongly with RBCs
Jk is common.
the serum of glycoprotein 77% of weeks. that carry a double dose of the
Jk(a-b+) -IgG (antiglobulin reactive)
Mrs. Kidd, is a urea whites. antigen and may not react with
whose infant transporter Jk(a-b-) b
-IgM (partly) Jk(a+b+) RBCs.
Jk - -made in response to pregnancy or
had HDFN. inherited as Resistant to lysis by 2M urea. Enzyme JK Ags Antibody reactivity can also be
- transfusion.
codominant Recessive Jk -lack Jka, Jkb, and the common Papain X enhanced by using LISS or PEG
antigen Jk3.
-57% of -bind complement
alleles. blacks and Ficin X (to promote IgG attachment).
-most abundant among
Chloroq -titer of Anti-Jka or Anti-Jkb quickly In vitro hemolysis can
Polynesians, identified in only 28% of X
uine declines in vivo. (decline in antibody sometimes be observed with
Filipinos, Indonesians, Chinese whites reactivity and the difficulty in detecting
&Japanese Kidds are not very DTT X enzyme-treated RBCs if serum
Kidd antibodies are reasons why they is tested; antigen dose may
-studies on two individuals immunogenic. AET X
with this phenotype showed a are a common cause of HTRs, especially influence this hemolytic
Glycine- of the delayed type.)
marked defect in their ability to activity.
concentrate urine. acid X
-most Jk(a–b–) nulls are EDTA Anti-Jk3
homozygous for the rare -IgG antiglobulin-reactive antibody that
“silent” allele Jk. looks like an inseparable anti-JkaJkb.
Dominant association with a dominant
-individual making the antibody will
Jk (In) gene In(Jk), for“inhibitor,”
shows dominant pattern of type Jk(a–b–).
inheritance within -associated with severe immediate and
a Japanese family analogous to delayed HTRs and with mild HDFN.
the inhibitor gene responsible Autoantibodies with Kidd specificity
for the dominant type Lu(a–b–) -rare
phenotype in the Lutheran -associated with autoimmune
 blood group system. haemolytic anemia.
-Individuals with the dominant
type Jk(a–b–) phenotype do
not make anti-Jk3.

The Diego (010) System

Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression at Antigen Antibodies Demonstrates Dosage Clinical Significance
Discoverer Found in -Antigen Birth Distribution: Complement Binding
Enzymes Plasma/RBC
DI DI (SLC4A1) Yes 22 expressed on RBCs of -Sometimes IgM -Both anti-Dia and anti-Dib
gene Dia/Dib newborns -usually IgG have caused HTRs and
named after the Wra/Wrb Dia -rare in most populations -reactive in the indirect HDFN.
first antibody Chromosome Wu/DISK but is polymorphic in Enzyme DI Ags antiglobulin test. -Anti-Wra has caused
maker in a 17: 17 low-prevalence people of Mongoloid
Venezuelan Band 3 and antigens Ficin X severe HTRs.
ancestry. Papain X Autoanti-Wrb is relatively
family during an Diego antigens -South American Indians,
investigation of are located. (except common in
the prevalence of Dia as
HDFN. Bpa ) the serum of patients with warm
high as 54%.
-present in the North and DTT X autoimmune haemolytic anemia.
Central American native Glycine- X
Localization of Dia and populations acid
Dib antigens to band 3 Dib Generally greater than 99% EDTA
enabled many low- but is 96% in Native
prevalence antigens to Americans.
be assigned to the
Wra low-prevalence antigen
Diego system: Wda, Rba, Wrb -Expression of Wrb requires
WARR, ELO, Wu, Bpa, the presence of both band 3
Moa, Hga, Vga, Swa, and a normal GPA of the
BOW, NFLD, Jna, KREP, MNS blood group system.
-GPA-deficient RBCs are
Tra, Fra, and Sw1.
Wr(a–b–).

The Yt (011) System

 Symbol Gene Antithetical -No. of Antigens RBC Antigen Antigen Distribution: Antibodies Demonstrates Clinical Significance
Discoverer Found in -Antigen Expression at Birth Plasma/RBC Complement Binding Dosage
Enzymes
YT YT (ACHE) gene Yes 2 -developed at birth -expressed more weakly on
a cord RBCs than on adult a b
Yt –high prevalence antigen in all Anti-Yt Anti-Yt
first antibody maker Chromosome 7 populations. RBCs
Enzyme YT Ags reasonably poor
and used the last b  - are absent from RBCs of
Three phenotypes are Yt -low prevalence antigen Ficin immunogenic immunogen
letter “t” in the  people with paroxysmal
observed: -found in about 8% of whites and 21% Papain -IgG
patient’s last name,  nocturnal
which wasCartwright Yt(a+b–) Common to 26% of Israelis, but not found in DTT -stimulated by pregnancy or
hemoglobinuria (PNH) III
Apparently Japanese. Glycine- X transfusion.
Yt(a+b+) Common acid
“why T” became “Yt.
Yt(a–b+) rare EDTA

The Xg (012) System

Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression at Antigen Distribution: Antibodies Demonstrat Clinical Significance
Discoverer Found in -Antigen Birth Plasma/RBC Complement Binding es Dosage
Enzymes
XG XG gene 2 -Cord RBCs express Xga a IgG
-9,000 copies of Xg
MIC2 (CD99) a weakly.
Xg per RBC
-discovered in 1962 in the serum of - -Weak expression of Xga is Anti-Xga has not been implicated in HDFN or as
a multiply transfused man. Chromosome CD99 a cause of HTRs.
a
seen on RBCs from some
-antigen Xg expression was X -known as 12E7 and MIC2 Two CD99– Japanese individuals have been
adult females, but weak found with alloanti-CD99.
controlled by an X-linked gene. The
expression on RBCs from
antigen was named after the X Xga has a phenotypic relationship to
CD99 adult males is rare.
chromosome and g for “Grand
Rapids,” where the patient was Enzymes XG Ags
Ficin 
treated
Papain 
DTT X

The Scianna (013) System

Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression at Antigen Distribution: Antibodies Demonstrates Clinical Significance
Discoverer Found in -Antigen Birth Plasma/RBC Complement Binding Dosage
Enzymes
SC SC (ERMAP) Yes 7 The antigens are expressed The antigens are IgG Only mild HDFN has
-named after the first antibody on cord RBCs. expressed on cord Reactive in antiglobulin test. been reported,
maker. In 1962, a new high Chromosome 1 Sc1/Sc2 Sc 1 RBCs. except for one severe
prevalence antigen was named Sm; 1 -Alloantibodies to Scianna antigens are rare case for anti-Sc4, for
Sc 2 Enzymes Sc Ags
year later, a new low prevalence -product of the gene is The low-prevalence -Autoantibodies to Sc1 and Sc3 have been which
Sc 3 Ficin X
a
antigen, Bu was found. erythroid membrane antigen Rd became reported. the baby required
Sc4. Sc5 (STAR), Sc6 Sc 4 Papain X
exchange
-individual in the Marshall Islands in associated protein DTT Slightly 
(SCER), and Sc7 Sc 5 transfusion.
the South Pacific was found with anti- (ERMAP), which is an
(SCAN) are all high- Sc 6
Sc3 RBC adhesion.
protein. prevalence antigens Sc 7
 The Dombrock (014) System
Symbol Gene Antithe -No. of Antigens RBC Antigen Expression at Antigen Antibodies Demonstrat Clinical
Discoverer Found in tical -Antigen Birth Distribution: Complement Binding es Dosage Significance
Enzymes Plasma/RBC
DO DO gene Yes 7 present on cord RBCs -present on cord a b a
Anti-Do and Anti-Do Anti-Do and
Hy RBCs b
-named for the first antibody maker, Chromosome 12 a -Absent from PNH anti-Do have
Jo Enzyme DO Ags -IgG caused
Mrs. Dombrock, found in 1965. Gy(a–) phenotype is the Dombrock null Ficin X III RBCs. -Reacting optimally with enzyme
Dombrock antigens are delayed HTRs
carried on a mono-ADP- -Doa and Dob antigens Papain X treated RBCs but no
ribosyltransferase 4 (ART4) -considered to be poor immunogens Glycine- X clinical HDFN.
attached to the RBC a
Gy -highly immunogenic. acid EDTA
membrane by a GPI 
anchor. Gy(a–) RBCs or Hy– RBCs are also Jo(a–) 0.2 M DTT

The Colton (015) System

Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression at Antigen Distribution: Antibodies Demonstrates Clinical Significance
Discoverer Found in -Antigen Birth Plasma/RBC Complement Dosage
Enzymes Binding
CO CO gene (AQP1) Yes 4 Expressed on RBCs of Colton antigens are -IgG Anti-Coa is often seen as a
Coa/Cob Coa newborns carried on an integral -Enhanced with single specificity and
-named in 1967 for the first antibody Chromosome 7 Cob membrane protein, enxyme-treated
has been reported to cause
maker; it should have been named RBCs.
Co3
Enzyme Co Ags
aquaporin 1 (AQP1), HTRs and HDFN.  Anti-Cob
Calton, but the handwriting on the Colton antigens are - present on all RBCs except those of the very which accounts for appears more often with
tube was misread carried on an integral Ficin X
rare Co(a–b–) phenotype 80% of water
membrane Papain X other specificities but has
Co4 readsorption in the
protein, aquaporin 1 Chloroqui X also caused HTRs and mild
high-prevalence antigen, has been identified
ne kidneys. HDFN.
(AQP1), on RBCs from two individuals with the Co(a–
DTT X -The glycoprotein Anti-Co3, which reacts
b–) phenotype.
crosses the RBC with all Co(a+) and Co(b+)
membrane multiple RBCs, has been reported to
times. cause severe HDFN.

The Landsteiner-Wiener (016) System

Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression Antigen Antibodies Demons Clinical Significance
Discoverer Found in -Antigen at Birth Distribution: Complement Binding trates
Enzymes Plasma/RBC Dosage
LW LW gene 3 Anti-LW reacts Anti-LW usually reacts depressed during pregnancy
Chromosome 19 a ab b equally well with strongly with D+ RBCs and lymphoma and
LW , LW , and LW
-had its origins along with the Enzyme LW Ags cord RBCs weakly (and sometimes leukemia.
discovery of the D antigen of the Rh Intracellular adhesion LW
a
LWb LW
ab
 regardless of their not at all) with D– RBCs
DTT
blood group system. molecule 4 (ICAM-4) D type. from adults, and not at all Autoanti-LW is also common
Glycine- X
-Landsteiner and Wiener reported carries the LW antigen in serum from patients with
acid EDTA with Rhnull RBCs.
that an antibody produced in warm autoimmune
rabbits (and later, guinea pigs) -are - low --are common, hemolytic anemia
after injection with RBCs of common, prevalence high-prevalence
rhesus monkeys reacted with 85% high- - found in antigens Many patients with anti-LW
prevalence less than -was originally have successfully been
of human RBCs.This antibody was
antigens 1% of most defined by the transfused
called anti-Rh for anti-rhesus.
Europeans antibody made by with D– RBCs.
but in 6% of an individual with
Finns. an inherited LW(a–
b–) phenotype.

The Chido/Rodgers (017) System

Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression at Antigen Distribution: Antibodies Demonstrates Clinical Significance
Discoverer Found in -Antigen Birth Plasma/RBC Complement Dosage
Enzymes Binding
CH/RG CH/RG (C4A,C4B) 9 not intrinsic to the RBC IgG clinically
gene Ch1 to Ch6, Rg1, and Rg2 are all highprevalence membrane. insignificant for
named after the first two WH has a prevalence of about 15% Enzyme CH/RG Ags transfusion
antibody producers, Ch for Chido Chromosome 6 Ch is present in 96% to 98% of most Ficin  fourth component of
and Rg for Rodgers. populations. Papain  complement (C4), and
The C4 glycoprotein Rg is present in 97% to 98% of most Glycine- X are adsorbed onto
Characterized as nebulous has two isoforms: populations acid EDTA RBCs from plasma
because antigen strength on C4B carries the Ch DTT X
different samples of RBCs was antigens and C4A
variable.It was also appreciated expresses Rg
that both anti-Ch and anti-Rg antigens.
could be neutralized by plasma.

The Gerbich (020) System

Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression at Antigen Distribution: Antibodies Demonstrates Clinical Significance
Discoverer Found in -Antigen Birth Plasma/RBC Complement Dosage
Enzymes Binding
GE GE(GYPC) gene 11 Gerbich antigens are RBCs of the Gerbich or IgG
Six high-prevalence Gerbich antigens expressed at birth. Leach phenotypes have IgM (some)
named in 1960 after Mrs.Gerbich, the Chromosome 2 (Ge2, Ge3, Ge4, GEPL, GEAT, and GETI) weak expression of Kell
first antibody producer. Five low prevalence antigens blood group antigens, Anti-Ge2 is the
Enzyme GE Ags
The antigens are carried on (Wb, Lsa, Ana, Dha, and GEIS) most common of
Ficin (Ge2 &
sialoglycoprotein structures the Gerbich
 GPC and Ge4) antibodies
GPD. X (Ge3)
Papain  (Ge2 &
The glycoproteins help to The Gerbich phenotype is polymorphic Ge4)
maintain the RBC membrane in certain areas of Papua, New Guinea. Glycine- X
integrity through interaction - found among Europeans, Africans, Native acid EDTA
with protein band 4.1, and Americans, Japanese, and Polynesians DTT X
about 135,000 copies of GPC and because they are rich in sialic
50,000 copies of GPD per RBC acid, they contribute to the net
negative charge of the RBC
membrane (as do GPA and GPB
of the MNS system).

The Cromer (021) System

Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression at Antigen Distribution: Antibodies Demonstrates Clinical Significance
Discoverer Found in -Antigen Birth Plasma/RBC Complement Dosage
Enzymes Binding
CROM CROM (CD55) gene Tca, Tcb, and Tcc, are 16 DAF is strongly IgG PNH III RBCs are
antithetical 13 high-prevalence antigens and 3 low expressed on placental deficient in DAF
Antibody was found in a black Chromosome 1 prevalence antigens Enzyme CROM tissue and will adsorb Anti-Cra and so they also lack
prenatal patient, Mrs. Cromer, a
Tc is the high- Ags Cromer antibodies anti-Tca have Cromer antigens.
that reacted with all RBCs except antigens of the Cromer The Cr(a–) phenotype is typically found in Ficin X
prevalence antigen been implicated
system are carried on decay blacks and is not found in whites
her own and two siblings. It was and the other two Papain X in HTRs
originally thought her antibody accelerating factor (DAF, are low prevalence - 
Dra antigen is of high prevalence;
recognized an antigen antithetical CD55), a complement Chymotrypsin Anti-IFC is the
Dr(a–) RBCs have weakened expression of
a regulatory protein. antibody made
to Go of the Rh blood group all other high prevalence Cromer antigens 
due to a markedly reduced copy number of DTT
system. The antibody was named by individuals
DAF. Glycine-acid X
anti-Cra in 1975. with the Cromer
EDTA
null Inab
phenotype

The Knops (022) System

Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression at Antigen Antibodies Demons Clinical
Discoverer Found in -Antigen Birth Distribution: Complement Binding trates Significance
Enzymes Plasma/RBC Dosage
KN KN(CR1) gene Yes 9 Weakly expressed on cord -IgG a
anti-Kn is
Chromosome 1 b b a -reactive in the antiglobulin
low-prevalence antigens Kn and McC Sl is RBCs and weaken upon frequently
Named after Mrs. Knops, the first a b test, but are clinically insignificant
Kn /Kn present on RBCs of only about 60% of African storage of adult RBCs (e.g., found in
antibody maker. the antigens were shown to be a b Americans. for both transfusion and HDFN. multiply
McC /McC older units of blood).
located on complement transfused
 receptor 1 (CR1)
Sl /Vil
a Enzyme KN Ags individuals and
The “Helgeson phenotype” represents the CR1 binds the complement multispecific
serologic null phenotype for the Knops blood Ficin  component fragments C3b and C4b sera; anti-Sla is
group Papain  and processes immune complexes more
DTT  for transportation to the liver and frequently
Glycine-acid X spleen and subsequent clearance found in blacks.
EDTA from the circulation.CR1 has also
been identified as a receptor for
several pathogenic organisms.

The Indian (023) System

Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression at Antigen Antibodies Demonstrates Clinical Significance
Discoverer Found in -Antigen Birth Distribution: Complement Binding Dosage
Enzymes Plasma/RBC
IN IN (CD44) gene Yes 4 Ina and Inb are weakly -IgG The extremely rare
expressed on cord RBCs -reactive in the In(a–b–) phenotype
named because the first In(a+) Chromosome 11 b CD44 is reduced antiglobulin has been found
In is the
individuals were from India. antithetical high- on RBCs of test and they do not in only one individual
These and two other Enzyme IN Ags dominant type bind complement. who presented with
prevalence
high-prevalence antigens are antigen. Lu(a–b–) congenital
Ficin 
located on CD44, an adhesion individuals Positive DATs dyserythropoietic
Papain 
molecule. but no clinical HDFN anemia and whose
DTT  have been reported for RBCs also typed Co(a–
Glycine-acid X anti-Ina and b–)
EDTA
anti-Inb
Decreased cell survival
with anti-Ina and an
immediate HTR due to
anti-Inb

The Ok (024) System

Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression at Antigen Antibodies Demonstrates Clinical Significance
Discoverer Found in -Antigen Birth Distribution: Complement Binding Dosage
Enzymes Plasma/RBC
OK OK (BSG) gene 3 Oka is well developed on IgG Anti-Oka has not
RBCs from newborns reactive in the been reported to
Anti-Oka was identified in 1979 Chromosome 19 antiglobulin test cause HDFN.
and was named after the Enzyme OK Ags
antibody maker, Mrs. Kobutso. OK antigens are carried on
Because Ko was already in use, CD147, a member of the Ficin X The antibody caused
the first two letters were immunoglobulin Papain X reduced survival of
switched to Ok. Her parents were superfamily that mainly DTT X 51
Cr-labeled Ok(a+)
cousins from a small Japanese functions as receptors and Glycine-acid X RBCs injected into
EDTA
island. Two of three siblings of adhesion molecules the original antibody
the proposita were also Ok(a–) maker.

The Raph (025) System

Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression at Birth Antigen Antibodies Demonstr Clinical Significance
Discoverer Found in -Antigen Enzymes Distribution: Complement Binding ates
Plasma/RBC Dosage
RAPH RAPH (CD151) gene 1 Expressed on erythroid precursors MER2 is abundant one patient with the
MER2 of individuals with either MER2+ or on platelets antibody showed
The antigen name is derived from Chromosome 11 Three examples of alloanti signs of a transfusion
MER2– RBCs
monoclonal, and Eleanor MER2 were found in Jews reaction after
Roosevelt, the laboratory where MER2 is located on CD151, a originating from India and living transfusion with 3
the antibody was produced. MER2 expression decreases over in Israel; two were related. All
tetraspanin, which appears units
When MER2 was raised to system to be essential for the time with increasing maturation of three had end-stage renal
status, the system was named assembly of basement erythroid cells disease. A fourth example of
Raph for the first patient to make membranes in the kidney anti-MER2 was found in a
the alloanti-MER2. and skin. Enzyme RAPH Ags healthy Turkish blood donor.

Ficin X Those individuals who have

Papain X made anti-MER2 showed
-Chymotrypsin 
mutations in CD151

AET 
Trypsin 

The John Milton Hagen (026) System

Symbol Gene Antithetic -No. of Antigens RBC Antigen Expression at Antigen Antibodies Demons Clinical Significance
Discoverer Found in al -Antigen Birth Distribution: Complement Binding trates
Enzymes Plasma/RBC Dosage
JMH JMH (SEMA7A) gene 6 weakly expressed on cord DAT is positive and IgG JMH antibodies are

In 1978, a large number of samples Chromosome 15
with this antibody were characterized
and the antibody was named anti- JMH protein is the GPI linked
JMH for the first antibody maker, glycoprotein CD108 and the
John Milton Hagen gene SEMA7A
The I (027) System and i (collection number 207002) Antigen
Symbol Gene
Discoverer Found in
I IGNT (GCNT2) gene
Wiener and co- Chromosome 6
workers gave a
name to one I- formed by the action of
such agglutinin, glycosyl transferases
calling its
antigen I for I and i antigens are precursors
“individuality. for the synthesis of ABO and
Marsh and Lewis antigens, and thus they
Jenkins are internal structures on
reported finding these oligosaccharide chains
anti-i
ABH and Ii determinants on
the RBC membrane are
carried on type 2 chains that
attach either to proteins or to
lipids.
Antigen activity is associated
with a branched form of i
antigen. The IGnT (also
known as GCNT2) gene on
chromosome 6p24 encodes
JMH2 through JMH6; these are JMH RBCs some JMH is (predominantly IgG4 in acquired generally considered
variants associated with amino acid detected on the JMH-negative people) clinically insignificant
substitutions in the protein. Enzyme JMH patient’s RBCs
JMH is a high-prevalence antigen. Ags JMH levels decline during the later Rare examples of
JMH1 represents the antigen recognized Ficin  years of life, sometimes alloanti-JMH in
by antibodies made by individuals lacking  to the point of not being detected individuals whose RBCs
Papain
the JMH protein.  serologically. Once JMH express variant forms
DTT
expression is reduced, anti-JMH can of CD108 may be
Glycine-acid X
be made clinically significant.
EDTA
Antithe -No. of Antigens RBC Antigen Antigen Distribution: Antibodies Demonstrates Clinical Significance
tical -Antigen Expression at Birth Plasma/RBC Complement Binding Dosage
Enzymes
No 1 -At birth, infant I and i antigens are Anti-I
Both I and i are high- RBCs are rich in i found on the Anti-I -IgM -is not associated with HDFN because the
prevalence antigens, -During the first 18 membranes of -bind complement at room temparature antibody is IgM, and the I antigen is poorly
but are expressed in a months of life, the leukocytes -common autoantibody that can be found in expressed on infant RBCs.
reciprocal quantity and platelets in virtually all sera, although testing at 4°C and/or -Patients with M. pneumoniae often
relationship. of i slowly addition to RBCs against enzyme treated develop strong cold agglutinins with I
decreases as I -RBCs may be required to detect the reactivity specificity and can experience a transient
No true I– or i– increases until adult I and i have also been found in the serum of many normal healthy episode of acute abrupt hemolysis just as
phenotype. proportions are found in the plasma individuals, is benign—that is, not associated with in the infection begins
reached; adult RBCs and serum of adults vivo RBC destruction. to resolve.
i reactivity on RBCs is are rich in I and and newborns and in -Pathogenic autoanti-I (e.g., the type associated Ant-i
inversely proportional have only trace saliva, human milk, with cold agglutinin syndrome) typically consists of -Potent examples are associated with
to marrow transit amounts of i amniotic fluid, urine, strong IgM agglutinins with higher titers and a infectious mononucleosis (Epstein-Barr
time and RBC age in antigen. and ovarian cyst fluid. broad thermal range of activity, reacting up to 30° virus infections) and some
circulation. or 32°C lymphoproliferative disorders.
-Fetal, cord, and
IT Antigen adult i RBCs carry Ant-i -fairly rare antibody that gives strong reactions with IgM and IgG anti- IT reacting preferentially
-reacted strongly with predominantly cord RBCs and adult i RBCs and weaker reactions at 37°C have also been found in patients
cord RBCs, unbranched chains with adult I RBCs with warm autoimmune hemolytic
weakly with normal and have the i -Autoanti-i are IgM and react best with saline- anemia, with a special association with
adult RBCs, and most phenotype. suspended cells at 4°. not seen as a common Hodgkin’s disease
weakly with adult i antibody in healthy individuals.
RBC Conditions associated with increased i
 the -agglutinin recognizes Anti- -is commonly encountered in the serum of group A1 antigen on RBCs include those with
N- a transition state of i IH individuals. shortened marrow maturation time or
acetylglucosaminyltransferas into I and designated -reacts stronger with group O and group A2 RBCs dyserythropoiesis
e, which adds GlcNAc to form the specificity IT (T for than with group A1 RBCs.
the branches “transition”). Chronic dyserythropoietic anemia type II
-found in two or hereditary erythroblastic
The gene responsible for I populations: multinuclearity with a positive acidified
antigen (IGnT) codes for the Melanesians and serum test (HEMPAS) is associated with
branching enzyme. Yanomama Indians in much greater i activity on RBCs than
Venezuela control cord RBCs.

In Asians, the adult i phenotype has been

associated with congenital
Cataracts

The Gill (029) System

Symbol Gene Antithetical -No. of Antigens RBC Antigen Expression at Antigen Antibodies Demonstrates Clinical Significance
Discoverer Found in -Antigen Birth Distribution: Complement Binding Dosage
Enzymes Plasma/RBC
GIL GIL (AQP3) gene 1 One example of anti-GIL was found
following a hemolytic transfusion
Chromosome 9 The GILnull Enzyme RAPH Ags reaction.
phenotype results
found on the glycerol from a frameshift and Ficin Reactivity
transporter aquaporin a premature stop Papain Reactivity
3 (AQP3), a member of codon. DTT X
the major intrinsic Glycine- X
protein family of water acid EDTA
channels

The RH-Associated Glycoprotein (030) System

Symbol Gene Antithetic -No. of Antigens RBC Antigen Antigen Antibodies Demonstrates Clinical Significance
Discoverer Found in al -Antigen Expression at Distribution: Complement Binding Dosage
Birth Plasma/RBC
Enzymes
RHAG RHAG(RHAG) gene 2

newest blood Chromosome 6 Duclos (RHAG1)

group system

-The Rh associated
glycoprotein (RhAG) does not
have Rh blood group antigens
-its presence in a complex
with the Rh proteins is
essential for Rh antigen
expression.
a
Ol (RHAG2)
Absence of RhAG due to
inactivating mutations in
RHAG results in the Rhnull
phenotype; some missense
mutations in RHAG result
in the Rhmod phenotype