MIDTERM LEC: MINOR BLOOD GROUPS Flashcards by Chin Lys

Q

Location: anion exchange protein (AE-1) aka erythrocyte band 3

A

Diego Blood Group System

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Q

Linked to MONGOLIAN ANCESTRY

A

Diego Blood Group System

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Q

Mutation in AE-1 results to HS, Congenital Acanthocytosis, and Southeast Asian Ovalocytosis

A

Diego Blood Group System

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Q

Composition: 22 antigens, including three sets of antithetical pairs (Dia/Dib, Wra/Wrb, Wu/DISK) and 17 low-prevalence antigens

A

Diego Blood Group System

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Q

Naming: Named after the first antibody producer in a Venezuelan family during HDFN investigation.

A

Diego Blood Group System

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Q

Designation: DI, number 010 by ISBT

A

Diego Blood Group System

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Q

Function: Major integral RBC membrane glycoprotein, also known as AE1 or SLC4A1

A

Band 3 Protein

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Q

Structure: Crosses the membrane multiple times with amino- and carboxyl-terminal domains in the cytoplasm.

A

Band 3 Protein

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Q

Gene: SLC4A1, located at chromosome 17q21-q22.

A

Band 3 Protein

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Q

Antigens and Prevalence
Wra/Wrb:

A

Wra is low-prevalence; Wrb is high-prevalence and requires band 3 and GPA for expression.

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Q

Antigens and Prevalence
Dia/Dib:

A

Dia is rare but polymorphic in Mongoloid ancestry; Dib prevalence is generally >99%

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Q

Clinical Significance
Antibodies:

A

Usually IgG, reactive in indirect antiglobulin test. Anti-Dia, anti-Dib, and anti-Wra have caused HTRs and HDFN.

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Q

Clinical Significance
Low-Prevalence Antigens:

A

Little clinical data available except for anti-ELO, which has caused severe HDFN

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Q

Ytª and Ytb; found in acetylcholinesterase

A

Cartwright Blood Group System

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Q

Named in 1956 after Cartwright (“why T” became “Yt”)

A

The Yt System (011)

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Q

Two antigens: Yta (high prevalence) and Ytb (low-prevalence)

A

The Yt System (011)

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Q

ISBT designation: YT, system number 011

A

The Yt System (011)

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Q

Located on acetylcholinesterase (AChE) glycoprotein

A

Yt Blood Group System Antigen Characteristics

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Q

Gene on chromosome 7q22

A

Yt Blood Group System Antigen Characteristics

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Q

Variably sensitive to ficin and papain, sensitive to DTT, resistant to glycine-acid EDTA

A

Yt Blood Group System Antigen Characteristics

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Q

Present at birth, weaker on cord RBCs

A

Yt Blood Group System Antigen Characteristics

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Q

Absent in PNH III RBCs

A

Yt Blood Group System Antigen Characteristics

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Q

Ytb: ~8% in whites, 21-26% in Israelis, absent in Japanese

A

Yt Blood Group System Prevalence

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Q

Phenotypes: Yt(a+b–), Yt(a+b+), rare Yt(a–b+)

A

Yt Blood Group System Prevalence

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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