UNCOMMON BLOOD GROUPS P1 Flashcards
Composed of 22 antigens: three sets of high and low pairs of antithetical
antigens- Dia/Dib, Wra/Wrb, and Wu/Disk – and 16 low-prevalence antigens.
The Diego (010) System
The Diego (010) System was named after the first antibody maker in a __________ during an investigation of _____
Venezuelan family ; HDFN.
The Diego antigens are carried on _______ (RBC anion exchanger (AE1) or solute carrier family-4 anion exchanger, member 1 (SLC4A1)), a major integral RBC membrane glycoprotein with about 1 million copies per RBC.
band 3
The protein crosses the membrane multiple times, and both the amino- and carboxyl-terminal domains are in the cytoplasm.
The Diego (010) System
A large N-glycan on the fourth external loop carries over half the RBC A,
B, H, and I blood group Antigens.
The Diego (010) System
The Diego (010) System Reported in 1955, ______ had caused HDFN in a Venezuelan baby.
- ______ was described 2 years later.
anti-Dia ; Anti-Dib
______ is rare in most populations but is polymorphic in people of Mongoloid ancestry.
Dia
In South American Indians, the prevalence of Dia can be as high as _____
54%
____ is also present in the North and Central American native populations but is surprisingly rare in Canada and among the Alaskan Inuit.
Dia
The prevalence of Dib is generally greater than 99% but is ____ in Native Americans.
96%
The Dia/Dib polymorphism is located on the last (seventh) __________ of the protein.
extracellular loop
___, a low-prevalence antigen, and the antithetical, high-prevalence ____ are
associated with an amino acid substitution on the fourth external loop.
Wra ; Wrb
Expression of Wrb requires the interaction of band and normal GPA of the MNS blood group system. GPA-deficient RBCs are ______
Wr(a-b-).
_________ are expressed on RBCs of newborns.
Diego Antigens
the antigens are resistent to treatment with ficin and papain, DTT, and glycine-
acid EDTA, with exception of Bpa, which is sensitive to papain.
DIEGO ANTIGENS
Diego system antibodies are sometimes ____, but are usually ____, reactive in the antiglobulin phase of testing.
IgM ; IgG
Anti-Dia and Anti-Dib = Hemolytic Transfusion Reactions and HDFN
YES
_______ is a relatively common antibody in donors and patients; some are directly agglutinating, but most require the antiglobulin phase of testing to be detected.
anti-Wra
anti-Wra =
severe HTRs
________ in individuals with Wr(a-b-) RBCs have been described,
so information about clinical significance is insufficient.
Alloanti-Wrb
Autoanti-Wrb is relatively common in the serum of patients with ________
warm autoimmune hemolytic anemia.
Which was named in 1956 for the first antibody maker and used the
last letter “t” in the patient’s last name, which was Cartwright.
The Yt (011) System
____ is the high-prevalence antigen in all populations;
Yta
_____ is the low-prevalence antigen found in about 8% of whites and 21% to 26% of Israelis, but is not found in Japanese.
Ytb
Three phenotypes are observed in Yt System
The common Yt(a+b-) and Yt(a+b+) and the rare Yt(a-b+).
The Yt antigens are ______ and present an amino acid substitution on the glycosylphosphatidylinositol (GPI)- linked RBC glycoprotein acetylcholinesterase (AChE).
antithetical
______ is an important enzyme participating in neurotransmission, but the function of RBC-
bound AChE is not known. Chromosome 7
AChE
Yt antigens are variably sensitive to _____ and _____, are sensititive to ____,
and are resistant to ________ treatment.
ficin and papain ; DTT ; glycine-acid EDTA
the antigens are developed at birth but are expressed weaker on cord RBCs
than on adult RBCs and are absent from RBCs of people with _________________
paroxysmal nocturnal hemoglobinuria (PNH) III.
Anti-Yta and Anti-Ytb are ____ and are stimulated by _______ or ________
IgG ; pregnancy or transfusion.
_______ is not an uncommon antibody, so it appears that Yta is reasonably
immunogenic.
Anti-Yta
____ appears to be a poor immunogen, as the antibody is rare.
Ytb
Yt antibodies have not caused ______
HDFN
______________ may be helpful in determining clinical significance
of anti-Yta.
Monocyte phagocytosis (MMA)
_____ was discovered in 1962 in the serum of a multiply transfused man.
anti-Xga
the antibody detected an antigen with a higher prevalence in females than in
males.
The Xg(012) System
antigen Xga expression was controlled by an _______
X-linked gene
was named after the ______ and g for “_______” where the
patient was treated.
X chromosome ; Grand Rapids
there are two antigens in Xg system: _____ and _____
Xga and CD99.
the gene XG encoding Xga is located on the X chromosome at _______
Xp22.32
the gene responsible CD99, MIC2, is located on the X chromosome at ______ and on the Y chromosome ______
Xp22.2 ; Yp11
_____ became part of the Xg system because the MIC2 and XG genes are adjacent and homologous and two CD99- Japanese individuals were found with ______
CD99 ; anti-CD99
- Xga has a phenotypic relationship to Cd99:
- all Xg(a+) individuals have a ____ expression of CD99 on their RBCs,
- all Xg(a- ) females have _____ expression of CD99; however, 68% of Xg(a-) males have a expression and 32% have low expression of CD99.
high ; low
- The prevalence of Xga is ____ in males and ___ in females.
- because male have only 1 X chromosome, Xg(a+) males are hemizygotes.
- females, having 2 X chromosomes, can be homozygote or heterozygotes.
66% ; 89%
Weak expression of Xga is seen on RBCs from some adult ____, but weak expression on RBCs from adult males is _____
females ; rare
The antigen is sensitive to ____ and _____ but resistant to _____ treatment.
ficin and papain ; DTT
Xg Antibodies:
- Anti-Xga is usually _____;
- Some examples are ________
- Anti-Xga has not been implicated in _____ or as a cause of _____.
IgG; ; naturally occurring. ; HDFN or HTRs
- Named after the first antibody maker.
- In 1962, high prevalence antigen was named Sm; 1 year later, a new low
prevalence antigen, Bua, was found. After it was confirmed that these two
antigens were antithetical, the Scianna blood group was established in 1974, and
the two antigens were renamed Sc1 and Sc2, respectively. - The prevalence of Sc2 in Northern Europeans is 1% but is higher in the
Mennonite population.
The Scianna(013) System
The product of the gene is a protein called _______________
which is an RBC adhesion protein.
erythroid membrane-associated protein (ERMAP),
The Scianna(013) System Antigen
- In 1980, an individual in the Marshall Islands in the South Pacific was found with the ______
- The very rare Sc:-1,-2,-3 phenotype is the ________
- The Sc gene ERMAP is located on chromosome 1 at position ______
Sc;-1,-2. ; Scianna null type ; 1p34.2.
The low-prevalence antigen Rd became ____
Sc4.
_______, ______, and ______ are all high-prevalence.
Sc5 (STAR), Sc6 (SCER), and Sc7 (SCAN)
The Scianna antigens are resistant to _____ and _______
ficin and papain.
The antigen are also resistant to ________. except for Sc2, which has variable reactivity after DDT-treatment.
DTT treatment
The Scianna antigens are expressed on _______
cord RBCs
(Scianna Antibodies)
- Alloantibodies to Scianna antigens are _____, and little is known about their clinical significance.
rare
(Scianna Antibodies)
- They are usually ____ and reactive in the ________ phase of testing.
IgG ; antiglobulin
(Scianna Antibodies)
- None have been reported to cause a severe ____
HTR
(Scianna Antibodies)
- Only mild HDFN has been reported, except for one severe case for anti-Rd, for which the baby required _________
exchange transfusion.
(Scianna Antibodies)
- autoantibodies to ____ and ____ have been reported.
Sc1 and Sc3
Was named for the first antibody maker, Mrs. Dombrock (anti-Doa),
found in 1965.
The Dombrock (014) System
______, which recognizes the antithetical antigen, was identified in 1973.
Anti-Dob
The prevalence of the three resulting phenotypes, Do(a+b-), Do(a+b+), and Do(a-b+), varies in different populations. In whites, they are _____, _____, and ____ respectively.
18%, 49%, and 33%,
The high-prevalence antigens ____ and ____ were both described in 1967.
Gya and Hy
RBCs from whites who are Gy(a-) were found to be ____, but Hy- RBCs from blacks were weakly _____.
Hy- ; Gy(a+)
The high-prevalence antigen Joa was described in 1972, and the phenotypic relationship to Gya and Hy was later shown: Gy(a-) RBCs or Hy- RBCs were also _____
Jo(a-).
In 1995, it was reported that Gy(a-) RBCs were also _____
Do(a-b-).
The Gy(a-) phenotype is the _______
Dombrock null
The Dombrock system currently has five additional high-prevalence antigens:
DOYA, DOMR,
DOLG, DOLC, AND DODE.
The Dombrock antigens are carried on a _________ attached to the RBC membrane by a GPI anchor.
mono-ADP-ribosyltransferase 4 (ART4)
The gene ART4 encoding the Dombrock glycoprotein is located on _________ at position 12p13-p12.
chromosome 12
The Dombrock antigens are resistant to ficin, papain, and glycine-acid EDTA and are sensitive to 0.2 M DTT treatment.
yes
The antigens are present on cord RBCs, but are absent from ______ RBCs
PNH III
The ____ and _____ antigens are poor immunogens and antibodies are rarely found as single specificities
Doa and Dob
_____, is highly immunogenic.
Gya
_____ and _____ have caused delayed HTRs but no Clinical HDFN.
Anti-Doa and Anti-Dob
The ________ are usually IgG, reacting optimally with enzyme-treated RBCs.
These antibodies are usually weakly reactive and disappear.
Dombrock antibodies
- Consists of four antigens.
- The system was named in 1967 for the first antibody maker; it should
have been named “Calton”.
The Colton (015) System
The high- and low-prevalence antithetical antigens are _____ and _____
Coa and Cob
The third antigen, ____, is present on all RBCs except those of the very rare
Co(a–b–) phenotype.
Co3
____, a high-prevalence antigen, has been identified on RBCs from two
individuals with the Co(a–b–) phenotype.
Co4
The Colton antigens are carried on an integral membrane protein, ________
aquaporin 1 (AQP1).
The gene AQP1 is located on _______ at position 7p14
chromosome 7
The ___________ are expressed on RBCs of newborns and are
resistant to treatment with ficin and papain, chloroquine, and DTT
Colton antigens
usually IgG and are enhanced with enzyme treated RBCs.
(Colton antibodies)
(Colton antibodies)
______ is often seen as a single specificity and has been reported to cause HTRs and HDFN.
Anti-Coa
(Colton antibodies)
______ appears more often with other specificities but has also caused
HTRs and mild HDFN.
Anti-Cob
(Colton antibodies)
_____, which reacts with all Co(a+) and Co(b+) RBCs, has been reported to
cause mild HTR and severe HDFN.
Anti-Co3
- Had its origins along with the discovery of the D antigen of the Rh blood group system.
- In 1940, Landsteiner and Wiener reported that an antibody produced in rabbits (and later, guinea pigs) after injection with RBCs of rhesus monkeys reacted with 85% of human RBCs.
The Landsteiner-Wiener (016) System
- The two antibodies were later shown to be different in several studies;
⚬ in the 1960s, the human anti-Rh was renamed ____ (but called anti-Rho by some workers), and the rabbit anti-Rh was called ______ in honor of Landsteiner and
Wiener.
anti-D ; anti-LW
There are three LW antigens:
The first two, _____ and ____, are common, high-prevalence anti?gens,
and ___ is of low prevalence
LWa, LWab, and LWb.
LWa and LWab ; LWb
____ was originally defined by the antibody made by an individual with an in?herited LW(a–b–) phenotype.
LWab
The ISBT has used ____, ____., and _____ as the antigen numbers for LWa,
LWab, and LWb, respectively.
LW5, LW6, and LW7
The null phenotype is ________; in one individual who made anti-LWab (Mrs.
Big), this phenotype resulted from a 10-base pair deletion in exon 1 of an
LWa gene, which introduced a premature stop codon.
LW(a–b–)
phenotypic relationship between the D antigen and anti-LW.
yes
Anti-LW usually reacts strongly with ____ RBCs, weakly with ___ RBCs from
adults, and not at all with ____ RBCs.
D+ ; D– ; Rhnull
Distinguishing anti-LW from anti-D is most easily accomplished by testing
DTT-treated D+ RBCs:
⚬ the D antigen is not denatured by ____, so anti-D would still be detected;
⚬ however, LW antigen is destroyed by ____, so anti-LW would no longer
react. LW antigens are resistant to treatment of RBCs with ____ and _____
DTT ; DTT ; enzymes and
glycineacid EDTA.
The structure that carries the LW antigens is a glycoprotein known as
____________-
intracellular adhesion molecule 4 (ICAM-4).
The LW glycoprotein is part of the band 3/Rh macrocomplex;15 Rhnull RBCs lack the LW glycoprotein.
- The LW gene ICAM4 is located on chromosome __ at position 19p13.2
19
LW antigens may be depressed during pregnancy and in some diseases, such
as _______ and _____
Autoanti-LW made by these patients can appear to be an alloantibody;
as the antigen strength returns, the antibody diminishes.
lymphoma and leukemia.
- Autoanti-LW is also common in serum from patients with ________.
warm autoimmune
hemolytic anemia.
No anti-LW has been shown to cause serious HDFN or transfusion reactions.
Many patients with anti-LW have successfully been transfused with D–
RBCs.
yes
