MIDTERM LEC: MINOR BLOOD GROUPS Flashcards
Location: anion exchange protein (AE-1) aka erythrocyte band 3
Diego Blood Group System
Linked to MONGOLIAN ANCESTRY
Diego Blood Group System
Mutation in AE-1 results to HS, Congenital Acanthocytosis, and Southeast Asian Ovalocytosis
Diego Blood Group System
Composition: 22 antigens, including three sets of antithetical pairs (Dia/Dib, Wra/Wrb, Wu/DISK) and 17 low-prevalence antigens
Diego Blood Group System
Naming: Named after the first antibody producer in a Venezuelan family during HDFN investigation.
Diego Blood Group System
Designation: DI, number 010 by ISBT
Diego Blood Group System
Function: Major integral RBC membrane glycoprotein, also known as AE1 or SLC4A1
Band 3 Protein
Structure: Crosses the membrane multiple times with amino- and carboxyl-terminal domains in the cytoplasm.
Band 3 Protein
Gene: SLC4A1, located at chromosome 17q21-q22.
Band 3 Protein
Antigens and Prevalence
Wra/Wrb:
Wra is low-prevalence; Wrb is high-prevalence and requires band 3 and GPA for expression.
Antigens and Prevalence
Dia/Dib:
Dia is rare but polymorphic in Mongoloid ancestry; Dib prevalence is generally >99%
Clinical Significance
Antibodies:
Usually IgG, reactive in indirect antiglobulin test. Anti-Dia, anti-Dib, and anti-Wra have caused HTRs and HDFN.
Clinical Significance
Low-Prevalence Antigens:
Little clinical data available except for anti-ELO, which has caused severe HDFN
Ytª and Ytb; found in acetylcholinesterase
Cartwright Blood Group System
Named in 1956 after Cartwright (“why T” became “Yt”)
The Yt System (011)
Two antigens: Yta (high prevalence) and Ytb (low-prevalence)
The Yt System (011)
ISBT designation: YT, system number 011
The Yt System (011)
Located on acetylcholinesterase (AChE) glycoprotein
Yt Blood Group System Antigen Characteristics
Gene on chromosome 7q22
Yt Blood Group System Antigen Characteristics
Variably sensitive to ficin and papain, sensitive to DTT, resistant to glycine-acid EDTA
Yt Blood Group System Antigen Characteristics
Present at birth, weaker on cord RBCs
Yt Blood Group System Antigen Characteristics
Absent in PNH III RBCs
Yt Blood Group System Antigen Characteristics
Ytb: ~8% in whites, 21-26% in Israelis, absent in Japanese
Yt Blood Group System Prevalence
Phenotypes: Yt(a+b–), Yt(a+b+), rare Yt(a–b+)
Yt Blood Group System Prevalence
Yt(a–b–) not reported
Yt Blood Group System Prevalence
IgG, pregnancy/transfusion-stimulated
Anti-Yta and anti-Ytb
reasonably immunogenic, not uncommon
Yta
poor immunogen, rare antibody
Ytb
No reported HDFN cases
yt blood group antibodies
variable clinical significance for transfusion
Anti-Yta
Location: X chromosome short arm
Xg Blood Group System
Destroyed by enzyme treatment
Xg Blood Group System
Discovery: 1962, in a multiply transfused man
Xg Blood Group System
Name: Derived from X chromosome and “Grand Rapids”
Xg Blood Group System
Designation: XG, system number 012
Xg Blood Group System
Two antigens: Xga and CD99 (MIC2, 12E7)
Xg Blood Group System Antigens and Genes
Genes: Xga at Xp22.3, CD99 at Xp22.2
Xg Blood Group System Antigens and Genes
CD99 is part of Xg due to gene proximity and homology
Xg Blood Group System Antigens and Genes
Xga: 66% in males, 89% in females; males are hemizygotes
Xg Blood Group System
Expression and Characteristics
CD99 expression: High in Xg(a+) individuals, variable in Xg(a–) males
Xg Blood Group System
Expression and Characteristics
Both antigens escape X chromosome inactivation
Xg Blood Group System
Expression and Characteristics
Xga glycoprotein crosses RBC membrane once
Xg Blood Group System
Expression and Characteristics
Approximately 9,000 copies of Xga per RBC
Xg Blood Group System
Expression and Characteristics
Sensitive to ficin and papain, resistant to DTT
Xg Blood Group System
Antigen Sensitivity
Weak expression on cord RBCs and some adult females
Xg Blood Group System
Antigen Sensitivity
Anti-Xga: Usually IgG, some naturally occurring
Xg Blood Group System Antibodies
Not implicated in HDFN or HTRs
Xg Blood Group System Antibodies
Alloanti-CD99 found in two CD99-Japanese individuals
Xg Blood Group System Antibodies
Ag are expressed by the RBC adhesion protein ERMAP/Erythroid Membrane Associated Protein in humans
Scianna Blood Group System
ISBT Symbol: SC, number 013
Scianna Blood Group System
Antigens: Seven antigens, including Sc1 (formerly Sm), Sc2 (formerly Bua), Sc3, Sc4 (Rd), Sc5 (STAR), Sc6 (SCER), Sc7 (SCAN)
Scianna Blood Group System
Established: 1974, after confirming Sc1 and Sc2 as antithetical
Scianna Blood Group System
In PNH III - total absence of DO antigens
Dombrock Blood Group System
Location: Aquaporin-1 (AQP)
Colton Blood Group System
Location: Glycophorin C and D; associated with RBC membrane band 4.1 Leach Phenotype (GE: -2,-3,-4) presents with elliptocytosis
Gerbich Blood Group system
Found in C4 Complement Components
Chido/Rodgers Blood Group System
Location: Decay Accelerating Factor (CD55 or DAF)
Cromer Blood Group System
PNH III - total absence of Cromer antigen
Cromer Blood Group System
Location: Complement Receptor 1 (CR1 or CD35)
Knops Blood Group System
Decreased expression in cases of SLE and chronic cold agglutinin disease
Knops Blood Group System
Location: Semaphorin CDw108
John Milton Hagen (JMH) Blood Group System
Blood group associated with HLA (MHC Class III)
. Bennett-Goodspeed Antigens
Location: CD44 glycoprotein
Indian Blood Group System
Absence of JMH found in PNH III
John Milton Hagen (JMH) Blood Group System
Bga = HLA-b7; Bgb = HLA-B17; Bgc = HLA-A28
. Bennett-Goodspeed Antigens
Bg antibodies are destroyed by treating RBC antigens with chloroquine or EDTA glycine-HCL
. Bennett-Goodspeed Antigens
Sda is associated with mixed-field refractile agglutination
Sid
Neutralized by URINE from an Sda positive individuals and guinea pigs
Sid