UNCOMMON BLOOD GROUPS P2 Flashcards
Named after the first two antibody producers, Ch for Chido and Rg for Rodgers.
These two antibodies were described in 1967 and 1976.
The Chido/Rodgers (017) System
Serologically, they were both (The Chido/Rodgers (017) System) characterized as _______ because antigen strength on different samples of RBCs was variable.
NEBULOUS
It was also appreciated that both anti-Ch and anti-Rg could be neutralized by
_______
plasma
Ch and Rg antigens are not ______ to the RBC membrane.
Rather, they are on the _____ component of complement (C4), and are adsorbed onto RBCs from plasma
intrinsic ; fourth
C4 glycoprotein has two isoforms:
- C4B carries the Ch antigens
- C4A expresses Rg antigens.
The genes C4A and C4B at two closely linked loci located on chromosome __ at position 6p21.3 encode these isoforms.
6
The Chido/Rodgers system consists of nine antigens: ___ to ___, ____ and ___ are all high prevalence; ___ has a prevalence of about 15%.
Ch1 to Ch6, Rg1, and Rg2 ; WH
___ is present in 96% to 98% of most populations.
___ is present in 97% to 98% of most populations.
The antigens are destroyed by ficin and papain but are resistant to treatment with DTT and glycine-acid EDTA.
Ch; Rg
Anti-Ch and anti-Rg are usually ___ and react weakly, often to moderate or high titration endpoints.
IgG
Neutralization of anti-Ch and anti-Rg with pooled plasma is often used as part of the identification of these antibodies in a patient’s serum. ____ and ______ are clinically insignificant for transfusion.
Anti-Ch and anti-Rg
Was named in 1960 after Mrs. Gerbich, the first antibody producer.
The Gerbich (020) System
There are currently six high-prevalence Gerbich antigens (…….) and five low-prevalence antigens (…….)
Ge2, Ge3, Ge4, GEPL, GEAT, and GETI
Wb, Lsa, Ana, Dha and GEIS
- The antigen are carried on sialoglycoprotein structures GPC and GPD.
- There are about ______ copies of GPC, and ______ copies of GPD per RBC.
Gerbich Antigen ; 135,000 ; 50,000
- _____ and ____ = encoded by the GYPC gene, located on chromosome 2
at position 2q14.3. - ______ = Gerbich null phenotype
- All were English or North American.
- Ge null Phenotype are elliptocytic
GPC and GPD ; Leach type
Gerbich Negative Phenotypes:
Ge: -2, 3, 4
Type: Yus
Antibody: Anti-Ge2
Gerbich Negative Phenotypes:
Ge: -2, -3, 4
Type: Gerbich
Antibody: Anti-Ge2 or anti-Ge3
Gerbich Negative Phenotypes:
Ge: -2, -3, -4
Type: Leach Type
Antibody: Anti-Ge2 or anti-Ge3
___ phenotype has been found in Mexicans, Israelis, Mediterraneans.
Yus
The Gerbich phenotype is _______ in certain areas of Papua, New
Guinea, and has also been found among Europeans, Africans, Native
Americans, Japanese, and Polynesians.
- Expressed at birth.
- Are resistant to treatment with _____ and _______;
- ____ and ____ are ficin and papain sensitive,
- but ____ is ficin resistant.
polymorphic ; DTT and glycine-acid EDTA ; Ge2 and Ge4 ; Ge3
Gerbich Antibodies
- May be IgM, but most are ___.
- are sometimes clinically significant for transfusion and sometimes not.
- can be eluted from DAT+ cord bloods, but only 3 cases of serious HDFN due to anti-Ge3, and two were children of the same mother.
- babies born to mothers with anti-Ge3 should be monitored for several weeks after birth for anemia.
- _______ most common of the Gerbich antibodies. The antibody made by the Ge:-2,3,4 phenotype individuals
- but it is also more common antibody made by the Ge:-2,-3,4 phenotype and Ge:-2,-3,-4 phenotype individuals.
- ____ is less frequently made by the Ge;-2,-3,4 and Ge:-2,-3,-4
- ______ is a very rare antibody.
IgG ; Anti-Ge2 ; Anti-Ge3 ; Anti-Ge4
In 1965, an antibody was found in a black prenatal patient, Mrs. Cromer, that reacted with all RBCs except her own and two siblings.
- originally thought her antibody recognized an antigen antithetical to ____ of the Rh blood group system.
- the antibody was named ____ in 1975.
The Cromer (021) System ; Goa ; anti-Cra
- The antigens of the Cromer system are carried on __________ (DAF, CD55), a complement-regulator protein.
- The gene ____ is located on chromosome 1 at position 1q32.
- The glycoprotein encoded by the gene is attached to the RBC
membrane through a GPI linkage. - _______ are deficient in DAF so they also lack Cromer antigens.
- The Cromer system has ___ antigens.
- ___ have been classified as high-prevalence antigens and ___ low-prevalence antigens.
- All these antigens are absent from Inab phenotype RBCs, the Cromer null phenotype, which is very rare.
decayaccelerating factor ;
CD55 ;
PNHIII RBCs;
19 ; 15 ; 3
- ______ appears to be a novel Inab-like phenotype with a mutation that affects the expression of the Cromoer antigens.
- The _____ phenotype is typically found in blacks and is not found in whites.
- Three Cromer antigens, ___, ____, and ____, are antithetical;
- ___ is the high-prevalence.
- ____ (low prevalence) and ___ (high prevalence) are also antithetical.
- The ____antigen is of high prevalence
CROM (CROM19) ;
Cr(a-) ;
Tca, Tcb, and Tcc
Tca ;
WESa ; WESb;
Dra
- _____ RBCs have weakened expression of all other high-prevalence Cromer antigens
due to a markedly reduced copy number of DAF. - The Dr(a-) phenotype has been found only among jews from Bakhara and in Japanese.
- Cromer system antigens are resistant to treatment with ______ and ____ but are destroyed by _______, which is used to distinguish specificities in this from other blood group antibodies.
- Cromer antigens are weakened with ____ treatment and are resistant to ______
Dr(a-) ;
ficin and papain ; a-chymotrypsin
DTT ; glycine-acid EDTA.
(cromer antibodies)
- are usually ____, but do not cause HDFN.
- ____ is strongly expressed on placental tissue and will adsorb Cromer antibodies.
- _____ and _____ have been implicated in HTRs, but other examples of these
specificities have not been caused clinical reactions after transfusion of
incompatibilities units. - ______ is the antibody made by individuals with the Cromer null Inab phenotype.
IgG ;
DAF ;
Anti-Cra and Anti-Tca ;
Anti-IFC
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The Knops (022) System
- There are ____ antigens in knop blood group system.
- The system was established when the antigens were shown to be
located on _________ and was named after Mrs. Knops, the first antibody maker. - The gene CR1 is located on ________ at position 1q32.2.
nine ;
complement receptor 1 (CR1) ;
chromosome 1
(knops antigen)
- Except for the low-prevalence antigens ____ and ____, the Knops
antigens have a prevalence of more than ____ in most populations. - ____ is present on RBCs of only about 60% of African Americans.
- The antithetical pairs of antigens are ________________
- ______ are weakly expressed on cord RBCs and weaken upon
storage of adult RBCs. - The antigens are weakened by treatment with ____ and _____ and are
sdestroyed by ____; the antigens are resistant to ________ - The “__________” represents the serologic null phenotype for
the Knops blood group; - these RBCs type Kn(a-b-), Mc(a-), S1(a-), and Yk(a-) because of low copy
number of CR1, but they are not truly devoid of Knops antigens.
Knb and McCb ; 90% ;
S1a ;
Kna and Knb, McCa and McCb,
and S1a and Vil ;
Knops antigens ;
ficin and papain ; DTT ; glycine-acid EDTA ;
Helgeson phenotype ;
(knops antibodies)
- are usually ____, reactive in the ______ phase of testing, but are clinically insignificant for both ______ and ______
- ________ is frequently found in multiply transfused individuals and multispecific sera;
- _______ is more frquently found in blacks.
- _____ binds the complement component fragments C3b and C4b and processes immune complexes for transfusion to the liver and spleen and subsequent clearance from the
ciculation. - CR1 has also been identified as a receptor for several pathogenic organisms.
IgG ; antiglobulin ; transfusion and HDFN ;
Anti-Kna ;
Anti-S1a ;
CR1
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The Indian (023) System
was named because the first In(a+) individuals were from India.
* There are five antigens in the system.
The Indian (023) System
(The Indian (023) System Antigen)
- The antigen Ina was reported in 1973 and is present on RBCs of ___ of Indians,
____ of Iranians, and ____ of Arabs. - ____ is the antithetical high-prevalence antigen.
- these and three other high-prevalence antigens are located on ____, an
adhesion molecule. - the gene CD44 is located on __________ at position 11p13.
- _______ phenotype is extremely rare and found only on individual who
presented with congenital dyserythropoietic anemia and whose RBCs also typed Co(a-b-). - _____ and ____ are weakly expressed on cord RBCs and are sensitive to treatment with ficin, papain, and DTT but are resistant to glycine-acid EDTA
4% ; 11% ; 12% ;
Inb ;
Cd44 ;
chromosome 11 ;
In(a-b-) ;
Ina and Inb
(The Indian (023) System Antibodies)
- usually ____ and reactive in the _______ phase of testing and do not bind ______
- Positive DATs but no clinical HDFN have been reported for anti-Ina and anti-Inb;
- decreased cell survival with anti-Ina and an immediate HTRs due to anti-Inb have been reported.
IgG ; antiglobulin ; complement.
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The Ok (024) System
(The Ok (024) System)
- _____ high-prevalence antigens in the Ok system.
- was named after the antibody maker, ________.
- Two additional antigens, _____ and _____, recieved their names
becuase of the amino acid changes G to V and V to M, respectively.
Three ; Mrs. Kobutso ; OKGV and OKVM
(The Ok (024) System Antigen)
- The OK antigens are carried on ______, or ______, a member of the immunoglobulin superfamily that mainly functions as receptors and adhesion molecules.
- Basigin is a recerptor essential for invasion by ________.
- the OK gene BSG is on_______ at position 19p13.3.
- ____ is well developed on RBCs from newborns and is resistant to treatment
with ficin and papain, DTT, and Glycine-acid EDTA.
CD147 ; basigin
Plasmodium falciparum ;
chromosome 19 ;
Oka
(The Ok (024) System Antibodies)
- The original ______ was IgG, reactive in the antiglobulin phase of testing.
- The antibody caused reduced survival of Cr-labeled Ok(a+) RBCs injected into the original antibody maker, suggesting clinical significance; an in vitro monocyte-monolayer assay also predicted clinical significance.
anti-Oka ;
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The Raph (025) System
- The only antigen in the Raph system is _____
- The antigen name is derived from monoclonal, and Eleanor Roosevelt,
the laboratory where the antibody was produced. - MER2 is encoded by the gene _____ located on ________ at
position 11p15.5.
MER2 ; CD151 ; chromosome 11
(Raph Antigen)
- The polymorphism identified by the monoclonal antibodies indicated that 8% of the English blood Donor population is MER2-.
- MER2 is abundant on ______ and is expressed on _____ precursors of
individuals with either MER2+ or MER2- RBCs. - People whose RBCs type MER2- have ______, and those individuals who
have made alloanti-MER2 are true MER2-. - The antigen is resistant to treatment with ficin and papain but is sensitive to
treatment with trypsin, a-chymotrypsin, pronase, and AET.
platelets ; erythroid;
anti-MER2 ;
(Raph Antibodies)
- those individuals who have made anti-MER2 showed mutations in _____.
- little is known about the clinical significance of anti-MER2, but one patient with the antibody showed signs of a transfusion reaction after transfuions with 3 units.
CD151
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The John Milton Hagen (026) System
- _____ is a high-prevalence
- In 1978, a large number of samples with this antibody was
characterized and the antibody was named anti-JMH for the first
antibody maker, John Milton Hagen. - was established after it was shown that the JMH protein is the GPI-
linked glycoprotein CD108 and the gene SEMA7A was cloned. - The gene SEMA7A is located on _______ at position 15q22.9-q23.
JMH ;
chromosome 15
(JMH ANTIGEN)
- _________; these are JMH variants associated with amino acid substitution protein.
- _____ represents the antigen recognized by antibodies made by individuals lacking the JMH protein.
*_____ is weakly expressed on cord RBCs and is destroyed by treating RBCs with ficin and papain and DTT; - the antigen is resistant to treatment with glycine-acid EDTA.
JMH2 through JMH6 ;
JMH1 ;
JMH
(JMH ANTIBODIES)
- anti-JMH are not found in patients who lack the JMH protein or who have one of the variant JMH phenotypes.
- it is widely accepted that JMH levels decline during later years of life, sometimes to the point of not being detected serologically.
- once JMH expression is reduced, _______ can be made.
- This autoanti-JMH with a positive DAT has never been associated with autoimmune RBC destruction.
- Anti-JMH is usually IgG (predominantly _____ in acquired JMH-negative people).
- JMH antibodies are generally considered clinically insignificant.
- _______ in individuals whose RBCs express variant forms of CD108 may be
clinically significant.
anti-JMH ; IgG4 ; Alloanti-JMH
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Gill (029) System
- Anti-GIL was first identified in 1980, but the antigen was not raised to
system status until 2002 when it was shown that GIL is genetically
discrete from all other blood system. - Only one antigen, GIL.
The Gill (029) System
GIL ANTIGEN
- this antigen is found on the glycerol transporter _________, a
member of the major intrinsic protein family of water and glycerol channels. - the AQP3 is located on ________ at position 9p13.
- The ____ phenotype result from a frameshift and a premature stop
codon. - the antigen is resistant to DTT and glycine-acid EDTA treatment.
aquaporin 3 (AQP3) ;
chromosome 9 ;
GIL
RBCs of two babies born to mothers with anti-GIL had a positive DAT but no clinical HDFN.
GIL ANTIBODIES
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The Rh-Associated Glycoprotein (030) System
(The Rh-Associated Glycoprotein (030) System)
- _____ does not have Rh blood group antigens; however, its presence in
a complex with the Rh proteins is essential for Rh antigen expression.
RHAG
(RHAG ANTIGEN)
- RhAG is glycosylated on the first extracellular loop, carrying ABH antigens.
- RhAG is encoded by the gene RhAG, located on _________ at position
6p12.3. - Four antigens have been assigned to the RHAG system:
- _____ (high prevalence), ____ (very rare, low-prevalence), the high
prevalence ____ (for Duclos-like), and _______. - Homozygosity for the allele encoding Ola is associated with the ________
- Absence of Duclos and DSLK is associated with_______ or _____
chromosome 6 ;
Duclos ; Ola ; DSLK ; RHAG4
Rhmod
phenotype. ;
U- Rhnull or U- Rhmod
phenotypes.
(RHAG ANTIBODIES)
- Clinical significance of anti-Duclos, anti-Ola and anti-DSLK is unknown because of the rarity of the antibodies.
- _______ has caused a single case of severe HDFN, but significance for transfusion is
unknown.
Anti-RHAG4
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The FORS (031) System
- In 1987, the Forssman glycosphingolipid was first thought to be a subgroup of A called ______.
- In 2011, it was shown to be unrelated to the ABO system and renamed
_____ in honor of John Frossman, who first discovered the antigen.
Apae ;
FORS
(FORST ANTIGEN)
- There is only one antigen, _____.
- the gene located on _______ at position 9q34.13-q34.3.
- the GBGTI gene produces ______ , which is causes the formation of the Forssman glycolipid by the addition of N-acetylgalactosamine to the P
antigen. - _____RBCs expressing FORS1 do not react with Dolichos biflorus or
monoclonal anti-A. - FORS1+ donor RBCs may react in a crossmatch due to naturally occuring anti-FORS1 in the plasma of ABO-compatible FORS1-individuals.
- The ________ can serve as a receptor for pathogens such as
Escherichia coli, making one believe that human cells that express the
FORS1 may have an increased susceptibility to E. coli infection. - The antigen is resistant to DTT and glycine-acid EDTA.
FORS1 ;
chromosome 9 ;
glycosyltransferase ;
Group O ;
Forssman glycolipid ;
(FORS ANTIBODIES)
- Reactivity with anti-FORS1 is enhanced with _____ AND _____ treatment.
- The antibody is mostly ____ with optimal reactivity at room
temperature or 4 Degrees Celsius, with an unknown clinical
significance.
ficin and papain ;
IgM
was assigned a system symbol JR and number 32 in 2012
The JR (032) System
(JR ANTIGEN)
- ____ is a high-prevalence antigen in most populations;
- The _____ phenotype is found more commonly in Japanese.
- The gene ABCG2 is located on _________ at position 4q22.1.
- The encoded protei, _______, is a member of the adenosine triphosphate (ATP) binding cassette transporters broadly distributed throughout the body.
- There is only one antigen, ____, in the JR system.
- The antigen is fully developed at birth and is resistant to treatment with ficin
and papain, DTT, and glycine-acid EDTA.
Jra ;
Jr(a-) ;
chromosome 4 ;
ABCG2 ;
Jra ;
(JR ANTIBODIES)
- The first _______, named for the antibody maker Rose Jacobs, was described in 1970.
- Anti-Jra is usually _____.
- Clinical significance of anti-jra is not well established due to the rare occurence of the antibody.
- Anti-Jra caused severe HDFN in some cases, including two fatal cases of HDFN.
- Some patients with anti-jra have recieved _____ incompatible units for
transfusion and have no ill effect, but in other cases, incompatible transfucion
have resulted in HTRs.
anti-JRa ;
IgG;
Jr(a+)-
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The LAN (033) System
Was first named in 1961 after the first antigen-negative proband, LAngereis,
who made anti Lan.
The LAN (033) System
(LAN ANTIGEN)
- The _____ antigen, a high-incidence antigen, was raised from the 901 series of high-incidence antigens to system status in 2012 when the ABCB6-null allele was demonstrated with the Lan phenotype.
- The Lan- Phenotype occurs in about 1 in 20,000 people.
- The lan gene, ABXB6, located on ______ at position 2q36, encodes
another of the ATP-binding cassette transporters. - In individuals with the Lan- phenotype, other porphyrin transporters are
thought to compensate. - The protein is widely expressed in heart, Skeletal muscles, fetal liver, eye,
mitochondrial membrane, and Golgi apparatus. - The antigen is also DTT and EDTA/glycine resistant.
Lan ;
chromosome 2
(LAN ANTIBODIES)
- Cord RBCs have a stronger reaction with monoclonal anti-Lan than do adult
cells. - Reactivity with anti-Lan is resistant to ficin and papain treatment of RBCs;
- Anti-Lan is formed due to exposure via transfusion or pregnancy and has not
been knownto be naturally occuring. - Lan antibodies re IgG reacting at the antiglobulin phase of testing with some
antibodies known to bind complement. - Is considered clinically significant
- The first anti-Lan caused an immediate HTR.
- Lan- RBCs should be selected for transfusion.
- Anti-Lan has not been knwon to cause severe HDFN.
YES
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The Vel (034) System
_______ was first described in 1952 and was named after the individual in whom
the first antibody was identified.
Anti-Vel
(VEL ANTIGEN)
- ____, a high-prevalence antigen, was raised to system status in 2013 when
absence of SMIM1, a single-pass integral membrane protein, was shown to produce the Vel- phenotype. - There is one antigen, ____.
- Thegene SMIM1 is located on _______ at position 1p36.32.
- Vel antigen expression is weak on cord RBCs and can vary from person to
person on RBCs of adults. - The antigen is also resistant to glycine-acid EDTA and DTT treatment, trough
some examples of anti-Vel do not react with DTT-treated RBCs.
Vel ;
Vel ;
chromosome 1
(VEL ANTIBODIES)
- ______ is characterized by its ability to activate complement and cause in vitro
and in vivo hemolysis. - The antibody is most often ____ but can be IgM, and it has caused one case of severe HDFN in which the mother had previously been transfused.
- Reactivity with anti-Vrl can be enhanced with enzyme-treated RBCs.
Anti-Vel ;
IgG ;
