Diego Blood Group System

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Questions and Answers

Why is it crucial to study uncommon blood groups despite their infrequent occurrence in routine pre-transfusion testing?

  • Uncommon blood groups are always clinically insignificant.
  • Antibodies against these antigens can cause hemolytic reactions. (correct)
  • To identify common blood group antigens.
  • To ensure compatibility in routine transfusions.

A Venezuelan baby is diagnosed with Hemolytic Disease of the Fetus and Newborn (HDFN). Further testing reveals the presence of anti-Dia in the mother's serum. Which blood group system is implicated in this case?

  • YT System
  • Dombrock System
  • Colton System
  • Diego System (correct)

The Diego blood group system's antigens reside on which red cell protein?

  • Glycophorin A
  • Acetylcholinesterase
  • Aquaporin 1
  • Band 3 (AE1) (correct)

Why is the detection of the Diª antigen significant in determining ancestry?

<p>It is commonly found in Mongolians. (C)</p> Signup and view all the answers

The expression of Wra and Wrb antigens is dependent on the interaction of Band 3 with which other red cell protein?

<p>Glycophorin A (B)</p> Signup and view all the answers

What does the Wr(a-b-) phenotype indicate regarding Glycophorin A?

<p>Absence of Glycophorin A (D)</p> Signup and view all the answers

Which characteristic is associated with Diego antigens?

<p>Expressed on RBCs of newborns (A)</p> Signup and view all the answers

Which Diego system antibody is known to cause severe Hemolytic Transfusion Reactions (HTR)?

<p>Anti-Wra (D)</p> Signup and view all the answers

The YT blood group system derives its name from what source?

<p>The surname of the individual who first produced the antibodies. (A)</p> Signup and view all the answers

The antigens of the YT blood group system are housed on which enzyme bound in RBC membranes?

<p>Acetylcholinesterase (B)</p> Signup and view all the answers

What is a notable difference in the prevalence of the Yt antigen between different populations?

<p>It is more common in Israelis compared to white people. (B)</p> Signup and view all the answers

Which characteristic differentiates the reactivity of YT antigens on cord RBCs compared to adult RBCs?

<p>Cord RBCs express YT antigens more weakly. (C)</p> Signup and view all the answers

Which of the following is a characteristic of YT antibodies?

<p>They are mostly in IgG form. (D)</p> Signup and view all the answers

The Xg blood group system is associated with genes located on which chromosome?

<p>The X chromosome (D)</p> Signup and view all the answers

How does inheritance of Xg antigens differ between males and females due to its X-linked nature?

<p>Females can be heterozygous or homozygous for Xg. (A)</p> Signup and view all the answers

What is the gene product of the ERMAP gene, which is significant in the Scianna blood group system?

<p>Erythroid Membrane-Associated Protein (ERMAP) (D)</p> Signup and view all the answers

Which characteristic is associated with Scianna antigens?

<p>Can be weakened by DTT (A)</p> Signup and view all the answers

In the Dombrock blood group system, what is the clinical significance of anti-Gya?

<p>It is not commonly encountered since it is high prevalence (A)</p> Signup and view all the answers

What is a key similarity between Dombrock and Kidd system antibodies?

<p>Both disappear quickly, implicating a delayed HTR. (D)</p> Signup and view all the answers

The antigens of the Colton blood group system are carried on which protein?

<p>Aquaporin 1 (D)</p> Signup and view all the answers

How does chloroquine assist in blood banking procedures related to DAT-positive red cells?

<p>It removes IgG antibodies bound to red cells. (B)</p> Signup and view all the answers

A patient with a history of malaria requires a blood transfusion. The transfusion service uses chloroquine to treat the donor red cells. What is the purpose of this treatment?

<p>To remove interfering autoantibodies. (A)</p> Signup and view all the answers

The Landsteiner-Weiner (LW) blood group system antigens are dependent on what?

<p>The ICAM4 protein (D)</p> Signup and view all the answers

Which of the following antibodies is known to occur as an autoantibody?

<p>Anti-Wrº (B)</p> Signup and view all the answers

Which of the following antigens are sensitive to treatment by ficin and papain?

<p>Xg antigens (B)</p> Signup and view all the answers

Which of the following antigens is resistant to ficin and papain?

<p>Scianna antigens (A)</p> Signup and view all the answers

Which of the following antigens is sensitive to treatment by DTT?

<p>All of the above (D)</p> Signup and view all the answers

Which of the following antibodies may NOT cause HDFN?

<p>Anti-YTa (C)</p> Signup and view all the answers

A patient is typed and found to be Gy(a-). What does this result indicate?

<p>The patient has the null phenotype of the Dombrock system (D)</p> Signup and view all the answers

A patient is identified as Co(a-b-). What additional antibody are they most likely to produce?

<p>Anti-Co3 (B)</p> Signup and view all the answers

Individuals with the Wr(a-b-) phenotype lack what antigen?

<p>Wright hight antigen (C)</p> Signup and view all the answers

What is the function of the RBC adhesion protein Erythroid Membrane Associated Protein?

<p>Houses antithetical Scianna blood group antigens (C)</p> Signup and view all the answers

What determines YTa and YTb

<p>An amino acid substitution (A)</p> Signup and view all the answers

What effect does chloroquine have on red cells, if any?

<p>Resistant to ficin, papain, DTT, chloroquine (C)</p> Signup and view all the answers

Why are YT antigens not found in RBCs of paroxysmal noctural hemoglobinuria?

<p>Lack of Glycosylphosphatidylinositol (GPI) (D)</p> Signup and view all the answers

Anti-YTa antibodies:

<p>Are much more commonly found (C)</p> Signup and view all the answers

Why is Xga not implicated in HTR or HDN?

<p>Xga antigens are not immunogenic. (A)</p> Signup and view all the answers

In the Diego blood group system, how does the interaction between Band 3 and Glycophorin A impact Wr antigen expression?

<p>It is essential for the expression of Wra and Wrb antigens. (D)</p> Signup and view all the answers

How does the presence or absence of the Gya antigen relate to the overall Colton system phenotype in individuals with the null phenotype?

<p>Individuals lacking Gya are considered to be Do(a-b-) in the Dombrock system and typically exhibit an absence of Hy and Joa antigens. (D)</p> Signup and view all the answers

A researcher is studying the expression of YT antigens and observes weak expression on cord blood RBCs compared to adult RBCs. Which of the following best explains this observation?

<p>YT antigens are not yet fully developed at birth and require further enzymatic modification. (C)</p> Signup and view all the answers

How does chloroquine modify RBCs to assist in antibody identification in the blood bank, especially in cases of DAT-positive cells?

<p>Chloroquine removes HLA antigens and immunoglobulins attached to RBCs, reducing interference in antibody testing. (C)</p> Signup and view all the answers

A male patient who is Xg(a+) marries a female who is Xg(a-). What are the expected Xg phenotypes of their children?

<p>The daughters will be Xg(a+), and the sons will be Xg(a-). (C)</p> Signup and view all the answers

The LW blood group system antigens are carried on which molecule?

<p>Intracellular Adhesion Molecule 4 (ICAM-4) (A)</p> Signup and view all the answers

Which treatment effectively removes LW antigens from red cells?

<p>Dithiothreitol (DTT) (D)</p> Signup and view all the answers

How can anti-LW and anti-D antibodies be differentiated when both react with red cells?

<p>Using Dithiothreitol (DTT) to remove LW antigens (A)</p> Signup and view all the answers

Which of the following antigens are adsorbed onto the red cell membrane rather than being an integral part of it?

<p>CH/RG antigens (C)</p> Signup and view all the answers

The genes responsible for Chido/Rodgers antigens reside on which chromosome?

<p>Chromosome 6 (C)</p> Signup and view all the answers

Antigens from this blood group are known to be expressed at birth and are carried on Glycophorin C and Glycophorin D.

<p>Gerbich blood group (B)</p> Signup and view all the answers

What is the effect of ficin and papain enzymes on Ge2 and Ge4 antigens of the Gerbich system?

<p>Destruction (C)</p> Signup and view all the answers

Which characteristic is associated with the Leach phenotype in the Gerbich blood group system?

<p>Lack of all Gerbich antigens (C)</p> Signup and view all the answers

Which of the following antigens is NOT weakened by AET and DTT?

<p>Knops antigens (B)</p> Signup and view all the answers

Which blood group system is associated with Decay Accelerating Factor (DAF)?

<p>Cromer (B)</p> Signup and view all the answers

A patient with a Cromer null phenotype is likely to produce which antibody?

<p>Anti-IFC (D)</p> Signup and view all the answers

The antigens of the Knops blood group system are carried on which protein?

<p>Complement Receptor 1 (CR1) (D)</p> Signup and view all the answers

Which characteristic is associated with Knops system antigens?

<p>Weak expression on cord red blood cells (A)</p> Signup and view all the answers

What is a common characteristic of antibodies in the Knops blood group system?

<p>They are detected with AHG (A)</p> Signup and view all the answers

The CD44 molecule carries the antigens for which blood group system?

<p>Indian system (C)</p> Signup and view all the answers

Which of the following antigens are sensitive to ficin, papain, and DTT?

<p>Indian antigens (D)</p> Signup and view all the answers

What is the clinical significance of Anti-Inª antibodies in the Indian blood group system?

<p>Decreased RBC survival (D)</p> Signup and view all the answers

What is the primary function of Basigin protein (CD147) in the OK blood group system?

<p>Adhesion and receptor function (C)</p> Signup and view all the answers

Which characteristic is associated with OKª antigen?

<p>Well-developed in newborns (D)</p> Signup and view all the answers

The MER2 antigen belongs to which blood group system?

<p>RAPH (D)</p> Signup and view all the answers

MER2 antigen expression changes as the red cell matures in what way?

<p>It decreases (D)</p> Signup and view all the answers

Which treatment is MOST likely to destroy MER2 antigen?

<p>Trypsin (A)</p> Signup and view all the answers

Individuals negative for the MER2 antigen may produce which corresponding antibody?

<p>They typically don't produce the antibody (A)</p> Signup and view all the answers

What is the reported clinical significance of Anti-MER2?

<p>May cause HTR (B)</p> Signup and view all the answers

Which antigen is NOT resistant to ficin and papain?

<p>MER2 (A)</p> Signup and view all the answers

A patient with the VERY RARE null LW phenotype has?

<p>LW antibody in IgM form (B)</p> Signup and view all the answers

A patient that has severe anemia after birth, had an Anti-Ge3 antibody. What blood group system is applicable?

<p>Gerbich blood group (D)</p> Signup and view all the answers

Which antigen does not have expression expressed in cord cells?

<p>Cromer (A)</p> Signup and view all the answers

A weak Cromer antigen phenotype has?

<p>Weak number of all Cromer antigens (B)</p> Signup and view all the answers

What happens to the D antigen when differentiating between and anti-LW and anti-D antibody?

<p>D antigen is not destroyed by DTT (D)</p> Signup and view all the answers

How can CH/RG antigens strength differ on different RBCs?

<p>Antigen strength is going to differ on different RBCs (C)</p> Signup and view all the answers

The Gerbich Blood group system is rich in?

<p>Rich in Sialic acid (B)</p> Signup and view all the answers

Anti-Sla:

<p>Frequently found in the black population (D)</p> Signup and view all the answers

OK antigens are resistant to?

<p>All of the above (D)</p> Signup and view all the answers

If a patient has end stage renal disease, what antibody are they more likely to have?

<p>Anti-MER2 (A)</p> Signup and view all the answers

An individual with end stage renal disease tests positive for Anti-MER2 (IgG), and must have a blood transfusion, will cause?

<p>Might cause HTR (A)</p> Signup and view all the answers

A VERY RARE phenotype that is EXTREMELY rare and only found in one individual and presented congenital dyserythropoietic anemia is?

<p>In(a-b-) (C)</p> Signup and view all the answers

Why might the presence of Chido/Rodgers (CH/RG) antigens in plasma complicate antibody identification?

<p>The soluble antigens neutralize CH/RG antibodies, leading to false negatives. (D)</p> Signup and view all the answers

How does the expression of Knops system antigens typically change on red blood cells after storage?

<p>Antigen expression decreases due to degradation. (B)</p> Signup and view all the answers

In the Gerbich blood group system, how does the absence of Glycophorin C and D in the Leach phenotype impact red cell structure?

<p>It weakens the red cell membrane, potentially leading to elliptocytosis. (C)</p> Signup and view all the answers

A patient with a known anti-Ykª antibody requires a blood transfusion. What is the MOST appropriate course of action for the transfusion service?

<p>Provide antigen-negative, crossmatch-compatible units. (B)</p> Signup and view all the answers

How does the structural alteration in the Basigin protein (CD147) that defines the OKVM antigen differ from that of the OKGV antigen?

<p>OKVM involves a substitution to methionine, while OKGV involves a substitution to valine. (C)</p> Signup and view all the answers

What is the protein CD108 associated with in the JMH system?

<p>Where JMH antigens reside. (D)</p> Signup and view all the answers

Why are anti-JMH antibodies considered clinically insignificant?

<p>They are commonly found in high titers but react weakly and do not typically cause HTR or HDFN. (B)</p> Signup and view all the answers

What is a unique characteristic of JMH antigen expression as individuals age?

<p>Expression tends to decline after the age of 50. (C)</p> Signup and view all the answers

What is a notable characteristic of the antibodies found in the Gill system?

<p>They are commonly IgG and cause HTR but not HDFN. (C)</p> Signup and view all the answers

What protein carries the antigens of the GIL blood group system?

<p>Aquaporin 3 (AQP3). (C)</p> Signup and view all the answers

What is crucial for the expression of Rh antigens D, C, and E?

<p>A complex with Rh proteins. (B)</p> Signup and view all the answers

What is the clinical significance of anti-Duclos in the RHAG system?

<p>Significance is still UNKNOWN in HTR and HDFN; antibodies are rarely encountered. (D)</p> Signup and view all the answers

What is the primary function of GBGT1 gene in the FORS system?

<p>Produces Glycosyltransferase, which forms Forssman glycolipid. (A)</p> Signup and view all the answers

What effect do ficin and papain enzymatic treatments have on FORS1 antigens?

<p>They enhance the expression of FORS1 antigens. (C)</p> Signup and view all the answers

An individual with a higher concentration of FORS1 antigen in their body may be more susceptible to what type of infection?

<p>Escherichia coli. (B)</p> Signup and view all the answers

What is the effect of treating the Jra with ficin, papain, DTT, and glycine-acid EDTA?

<p>Has no effect on Jra antigen expression. (A)</p> Signup and view all the answers

The ABCB6 gene product is the gene product responsible for which blood group system?

<p>LAN system. (D)</p> Signup and view all the answers

Which of the following is true regarding the expression of Lan antigen in different cell types?

<p>Widely expressed in muscle, heart, fetal liver, eye, and mitochondrial membranes. (A)</p> Signup and view all the answers

What is the potential clinical implication of anti-Lan antibodies?

<p>Causes HTR and mild HDFN. (D)</p> Signup and view all the answers

What biochemic treatment is known to affect the Vel antigen?

<p>DTT. (A)</p> Signup and view all the answers

What is the clinical significance of anti-Vel?

<p>Causes HTR and severe HDFN. (A)</p> Signup and view all the answers

What role does MIRL (membrane inhibitor of lysis) play in complement regulation?

<p>Interferes with the formation of the membrane attack complex (MAC). (B)</p> Signup and view all the answers

What symptoms are likely to be exhibited by individuals with CD59 deficiency?

<p>PNH-like symptoms such as hemolysis, strokes and neuropathy. (D)</p> Signup and view all the answers

Which of the following genetic codes is the gene responsible for the Augustine System?

<p>SLC29A1 gene. (A)</p> Signup and view all the answers

Which treatments do NOT affect the Augustine blood group system?

<p>Ficin, papain, DTT, and glycine-acid EDTA. (C)</p> Signup and view all the answers

What are the clinical implications of Anti-Ata?

<p>Causes severe HTR and mild HDFN. (A)</p> Signup and view all the answers

Which of the following is a low-prevalence antigen in the AUG system, also known as Atª?

<p>AUG1. (D)</p> Signup and view all the answers

Which of the following antigens is commonly associated with paroxysmal nocturnal hemoglobinuria (PNH)?

<p>CD59.1 (B)</p> Signup and view all the answers

Which of the following treatments are utilized in order to remove HLA antigens found on the surface of RBCs?

<p>Chloroquine and glycine-acid EDTA. (B)</p> Signup and view all the answers

In what form will HLA Class I antigens will be found on RBCs?

<p>Immature form. (C)</p> Signup and view all the answers

Which of the following conditions is associated with antibodies against Bennett-Goodspeed antigen?

<p>Both B and C. (B)</p> Signup and view all the answers

What best describes ISBT blood group collections?

<p>Do not meet the standards to be called a system. (C)</p> Signup and view all the answers

What best describes the ISBT 700 series?

<p>Low prevalence antigens. (C)</p> Signup and view all the answers

What is the known percentage of the most random population that constitutes the 700 series?

<p>Found in 1% or less. (D)</p> Signup and view all the answers

Which statement accurately describes the ISBT 901 series antigens?

<p>High frequency antigens, more than 90% of the population is positive. (A)</p> Signup and view all the answers

Which phrase accurately describes how to find compatible units from the ISBT 901 series?

<p>Require more genetic studies in order to identify the genes that causes its expression. (A)</p> Signup and view all the answers

How does the amount of HLA antigens change on RBCs as they mature?

<p>Decrease and become undetectable. (B)</p> Signup and view all the answers

To the mature RBCs that contain HLA antigens, they are given another name. Those antigens are?

<p>All of the above. (D)</p> Signup and view all the answers

Which of the following are true regarding the Bennett-Goodspeed antigen

<p>All of the above. (D)</p> Signup and view all the answers

Anti-CD59.1 is found in?

<p>Patients. (C)</p> Signup and view all the answers

How does the expression of JMH antigens typically change as a person ages?

<p>The expression of JMH antigens decreases after 50 years of age. (B)</p> Signup and view all the answers

What is a notable characteristic of the anti-JMH antibodies in terms of clinical significance?

<p>Anti-JMH antibodies are clinically insignificant and do not typically cause Hemolytic Transfusion Reactions (HTR) or HDFN. (C)</p> Signup and view all the answers

The GIL antigen is carried on Aquaporin 3 (AQP3) and is enhanced with ficin and papain. What statement best describes this antigen?

<p>GIL belongs to the major intrinsic protein family of water and glycerol channels and are resistant to glycine-acid EDTA and DTT. (B)</p> Signup and view all the answers

What is the primary requirement for the expression of Rh antigens D, C, and E?

<p>The presence of the RHAG gene product in complex with Rh proteins (B)</p> Signup and view all the answers

A patient with CD59 deficiency is MOST likely to exhibit symptoms related to what condition?

<p>Paroxysmal nocturnal hemoglobinuria (PNH) (B)</p> Signup and view all the answers

Flashcards

RBC Antigen Diversity

RBCs have different antigens from different blood group systems, making each individual unique.

Why study Uncommon Blood Groups?

Important for gene production, red cell function, antigen prevalence, antibody detection, and clinical significance due to hemolytic reactions.

Clinically Significant Antibodies

Antibodies against uncommon blood group antigens that can cause hemolytic transfusion reactions and hemolytic disease of the fetus and newborn.

Diego Blood Group System History

Named after Venezuelan baby with HDFN in 1955, most people have Diᵇ, important for determining Mongolian ancestry.

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Gene responsible for Diego BGS

The SLC4A1 gene codes for a protein located on chromosome 17.

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Function of Band 3

Band 3 is involved in anion exchange (bicarbonate and chloride ions) and interactions with cytoskeletal proteins.

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Wright Antigen Expression

Wright antigens require Band 3 interaction with Glycophorin A; null phenotype is Wr(a-b-).

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Diego Phenotype Prevalence

Most common is Di(a-b+); rare is Di(a-b-), and not yet reported.

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Diego Antigen Characteristics

Expressed on newborns, resistant to ficin, papain, DTT, sensitive to papain, reacts at AHG.

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Anti-Dia, Anti-Dib Significance

Commonly causes HTR and HDFN.

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History of YT System

First antibody was found in someone named Cartwright.

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Function of AChE bound to RBC

The function of this enzyme is not known.

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AChE role in YT antigens

Housing the YT antigens. YT is present in 8% of white people and more common in Israelis (21-26%).

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Gene responsible for YT BGS

Resides in Chromosome 7.

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Difference between YTa and YTb

Based on amino acid substitution on Glycosylphosphatidylinositol(GPI).

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YT Antibodies Class

Mostly IgG (1 & 4).

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Anti-YTa Significance

YTª antigen is immunogenic vs. YTb.

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Xg Chromosomal Arrangement

Discovered genes for Xg blood group system are positioned adjacently in the X chromosome.

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Xg Antigen Frequency

Majority of females tend to have Xg antigen.

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Xg Inheritance Pattern

If a male is Xg positive and marries a female that is Xga negative, their daughters will be Xg (+) and their sons will be Xg (-).

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Xg Gene Dosage

They can be heterozygous/homozygous for Xg and MIC2 genes, where males are hemizygous.

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SC Gene Product

Erythroid Membrane-Associated Protein (ERMAP) found on Chromosome 1.

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Dombrock Antibodies Analogy

Shares similarities with Kidd Abs: titers don't last long and can cause delayed HDR.

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Dombrock Antibodies Reactivity

The antibodies go away just as fast as the titers went up implicating delayed HDR.

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History of Colton System

In 1967, Calton was misread.

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Function of Aquaporin 1

Integral protein responsible for 80% of the water reabsorbed in the kidneys.

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Chloroquine Use

Anti-malarial drug; Utilized in blood banks to FREE IgG (used to remove HLA-antigens present on the surface of the red cells).

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What is the LW gene product?

ICAM-4; housing 3 LW antigens

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What characterizes EDTA antibodies?

IgG, neutralized by plasma; clinically insignificant.

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What characterizes CH/RG antigens?

Two BGS combined because they reside on same protein; C4 complement is the carrier of CH/RG that are adsorbed.

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Which gene is responsible for the Gerbich System?

GYPC gene, residing on Chromosome 2

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What is the gene product of Gerbich System?

Glycophorin C and D, interacts with Protein 4.1 and P55 in the Red Cell membrane.

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Gerbich Antigens Enzyme Sensitivity?

Depending on the antigen, they could be Resistant/sensitive to specific enzymes

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Ge3 Enzyme Sensitivity

Ficin resistant

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Anti-Ge2 Characteristics?

Anti-Ge2 (most common), Mostly IgG, rarely IgM (RT, enhanced by AHG)

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What gene is responsible for Cromer System?

CD55 gene; Resides in Chromosome 1

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What is the gene product of Cromer?

Decay Accelerating Factor (DAF); Complementary regulatory protein

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Knops Antigens Characteristics

Weak expression on Cord RBCs and weakens upon storage; Weakened by ficin and papain

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Gene for the Knops System?

CR1 gene Residing on Chromosome 1

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Knops Antibodies Detection

Detected with AHG

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What gene is responsible for Indian System?

CD44 gene; Residing in Chromosome 11

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What is the gene product of Indian?

CD44 is an adhesion molecule

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What gene is related to OK System?

BSG Gene; Chromosome 19

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What is the gene product of OK System?

Basigin Protein (CD147)

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Characterize Anti-OKª

Anti-OKª is rare, Reactive on AHG test

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Which gene is associated with the RAPH system?

CD151 gene; Resides on Chromosome 11

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What does CD151 identify?

Identified as a Tetraspanin

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JMH1 Antigen

Antigen expressed on RBCs, placenta, thymus, and testes. Weak on cord RBCs. Declines with age.

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Anti-JMH

IgG antibody against JMH1 antigen, reactive at AHG phase and body temperature. Generally considered clinically insignificant.

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GIL Gene Product

AQP3; glycerol-transporter. Carries GIL antigen. Resistant to glycine-acid EDTA and DTT, enhanced by ficin and papain

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Anti-GIL

Commonly IgG, reactive at 37 degrees Celsius and enhanced by AHG. Can cause HTR, but rarely HDFN.

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RHAG Gene Product

RHAG (Rh-Associated Glycoprotein); Resides on Chromosome 6; No RHAG gene = Rh null phenotype

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RHAG Antigens

Duclos, Ola, DSLK; Requires complex with Rh proteins in order to express Rh antigens (D, C, E).

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Anti-RHAG Antibodies

Antibodies against RHAG antigens (Duclos, Ola, DSLK). Significance in HTR and HDFN is still UNKNOWN.

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FORS System Gene

GBGT1 gene. Produces glycosyltransferase that forms Forssman glycolipid FORS1.

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FORS1 Antigen

Addition of GalNac to P antigen; receptor for Escherichia coli; enhanced with ficin and papain.

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Anti-FORS1

IgM, reactive at 4 degrees Celsius; Unknown clinical significance

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JR System Gene

ABCG2 gene. Member of the ATP-binding cassette transporters.

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ABCG2 Function

Involved in multi-drug resistance of tumor cells; complications in chemotherapy.

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Jra Antigen

Fully developed at birth; resistant to ficin, papain, DTT. Named after Rose.

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Anti-Jra

IgG. Causes HDFN and HTR; uncommon.

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LAN System Gene

ABCB6 gene; functions in Heme synthesis.

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ABCB6 Function

ATP binding cassette transporter; transports heme and porphyrins into mitochondria.

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Lan Antigen

Present in red cells, heart, skeletal muscle, liver, etc. Resistant to ficin, papain, DTT and glycine-acid EDTA.

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Anti-Lan

IgG. Causes HTR and mild HDFN.

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Vel System Gene

SMIM1 gene, single-pass integral membrane protein on chromosome 1.

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Vel Antigen

Enhanced with ficin and papain; resistant to DTT and glycine-acid EDTA.

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Anti-Vel

IgG (most common) and IgM; causes HTR and severe HDFN; clinically significant. Difficulties in identifying its presence

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MIRL Function

Plays key role in protecting against complement-regulated hemolysis by interfering the membrane attack complex (MAC). Produced by CD59 gene.

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CD59 Gene

CD59 gene. Membrane inhibitor of lysis (MIRL) AKA (Glycosyl-phosphatidyl-inositol (GPI)-linked complement regulatory glycoprotein)

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CD59 Deficiency

Absence of CD59 means more prone to lysis because MAC cannot be inhibited. PNH-like symptoms such as Hemolysis, Strokes, Neuropathy.

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Augustine Gene

SLC29A1 gene.

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Augustine

Fully developed at birth; Resistant to ficin and papain, DTT, and glycine-acid EDTA

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Anti-Ata

Causes severe HTR and mild HDFN

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Study Notes

JMH System

  • ISBT symbol is JMH, and the number is 026
  • SEMA7A gene is responsible, residing on Chromosome 15
  • CD108 is where JMH antigens reside
  • JMH1 antigens are found on RBCs, placenta, thymus, and testes
  • JMH1 antigens are weak on cord RBCs
  • JMH1 antigens are sensitive to ficin, papain, and DTT
  • JMH1 antigens are resistant to glycine-acid EDTA
  • Additional 5 antigens are found in the JMH system
  • Anti-JMH (IgG) antibodies are against JMH1 but generally named Anti-JMH
  • Anti-JMH antibodies are reactive at AHG phase/body temperature, often found in high titer
  • Weakly reactive and clinically insignificant, and do not cause HTR or HDFN
  • Named after John Milton Hagen, the first person who produced the antibody
  • JMH1, JMH2, JMH3, JMH4, JMH5, and JMH6 antigens are present
  • JMH antigens are not exclusively found on RBCs
  • Can be found on placenta, thymus, and testes as well
  • JMH antigens expression increases further in adulthood
  • The number of antigens declines when you reach 50 years old and above
  • Anti-JMH antibodies aren't encountered in individuals who are JMH antigen negative naturally
  • These individuals don't have JMH antigens because of decline of antigen amount when they reach elderly stage
  • Weak reactions of JMH Antibodies are present
  • Placing JMH antigens found in the plasma does not neutralize Anti-JMH Abs in serum

Gill System

  • ISBT symbol is GIL, and the number is 029
  • AQP3 gene is responsible, residing on Chromosome 9
  • Glycerol-transporter Aquaporin 3 (AQP3) is the gene product that carries the GIL antigen
  • GIL antigen is carried by AQP3
  • GIL antigen is resistant to glycine-acid EDTA and DTT
  • GIL antigen is enhanced by ficin and papain
  • Member of the major intrinsic protein family of water and glycerol channels
  • Anti-GIL antibodies are commonly in the form of IgG, reactive at 37 degrees Celsius, and enhanced by AHG
  • Anti-GIL can cause HTR, but not HDFN, with only 1 reported case

Rh-associated Glycoprotein System

  • ISBT symbol is RHAG, and the number is 030
  • RHAG gene is responsible, residing on Chromosome 6
  • No RHAG gene results in Rh null phenotype
  • RHAG (Rh-Associated Glycoprotein) is the gene product
  • Antigens include Duclos, Ola, DSLK, and RHAG 4
  • Antibodies include Anti-Duclos, Anti-Ola, Anti-DSLK, and Anti-RHAG 4
  • Does not contain RH antigens itself, and need to be in complex with Rh proteins in order to express Rh antigens such as D, C, and E antigens

RHAG Antigens - High vs Low Prevalence

  • High Prevalence RHAG Antigens: Duclos, DSLK, RHAG 4
  • Low Prevalence RHAG Antigens: Ola

Antibodies of the RHAG System

  • Anti-Duclos, Anti-Ola, Anti-DSLK, and Anti-RHAG 4 are Antibodies of the RHAG System
  • Significance is still unknown in HTR and HDFN
  • Only 1 case where the antibody caused HDFN
  • Significance to transfusion (HTR) is unknown

FORS System

  • ISBT symbol is RHAG, and the number is 031
  • GBGT1 gene is responsible, residing on Chromosome 9
  • Product produces Glycosyltransferase and forms Forssman glycolipid that adds N-Acetylgalactosamine to the P antigen
  • The FORS1 antigen is also the receptor for Escherichia coli
  • Addition of GalNac (N-Acetylgalactosamine) to P antigen
  • Enhanced with ficin and papain and Resistant to DTT and glycine-acid EDTA
  • Assigned as Apae, mainly because of its immunodominant sugar
  • FORS1 antigen's structure is similar with A antigen, because of the N-acetylgalactosamine
  • Anti-FORS1 (IgM) reactive at 4 degrees Celsius with unknown clinical significance
  • The higher your FORS1 antigen present in the body, the more susceptible you are to E. coli infection
  • Forssman glycolipid not normally expressed in RBCs, but rather found in other tissues; whether healthy or malignant

JR System

  • ISBT symbol is JR, and the number is 032
  • ABCG2 gene is responsible, residing on Chromosome 4
  • Gene product is ABCG2 which is a member of the Adenosine triphosphate binding cassette transporters distributed all throughout the body
  • Involved in the multi-drug resistance of tumor cells and problems in chemotherapy
  • Antigen is Jra, which was discovered in 1970, named after Rose Jacobs
  • Fully-developed at birth
  • Resistant to ficin, papain, DTT, and glycine-acid EDTA
  • Anti-Jra (IgG) causes HDFN and HTR

LAN System

  • ISBT symbol is LAN, and the number is 033
  • ABCB6 gene is responsible, residing on Chromosome 2
  • The gene product is ATP binding cassette transporter, functions in Heme synthesis
  • Expressed on the red cell, but widely expressed in the heart, skeletal muscle, fetal liver, eye, mitochondrial membrane, and Golgi apparatus
  • Lan antigen is present approximately 99% of people
  • Lan antigen is resistant to ficin, papain, DTT, and glycine-acid EDTA
  • Anti-Lan (IgG) causes HTR and mild HDFN
  • With the ATP-dependent uptake of heme
  • porphyrins into mitochondria

Vel System

  • ISBT Symbol: VEL
  • ISBT Number: 034
  • SMIM1 gene is responsible, residing on Chromosome 1
  • Gene Product: SMIM1 is a single-pass integral membrane protein
  • Antigen: Vel with characteristics similar to what has been discovered, which Includes
    • Enhanced with ficin and papain
    • Resistant to DTT and glycine-acid EDTA
  • Antibodies: Anti-Vel (IgG [most common] and IgM) which contains characteristics similar to what was described
    • Causes HTR and severe HDFN
    • Clinically significant
    • Abs can activate complement and cause both in vivo and in vitro hemolysis
    • Reactivity varies from one red cell to another
    • Difficulties in identifying its presence

CD59 System

  • ISBT Symbol: CD59
  • ISBT Number: 035
  • Responsible Gene: CD59 gene
  • Gene Product: CD59 is a Membrane inhibitor of lysis (MIRL) AKA (Glycosyl-phosphatidyl-inositol (GPI) linked complement regulatory glycoprotein)
  • Antigens: CD59.1 with characteristics similar to what was mentioned
    • Enhanced by enzymes
    • Sensitive to DTT
    • Associated with PNH (PNH Pxs are deficient in ALL)
  • Properties of a GPI linked: The antigen binds with complement and they are all proteins
  • Cord RBCs tend to have a stronger reaction with Anti-Lan than do adult cells

MIRL

  • Protein for CD59
  • plays a key role in protecting against complement-regulated hemolysis by interfering the membrane attack complex (MAC) by inhibiting the binding of C8 and C9
  • This type of protein is produced by CD59 gene
  • Absence of CD59: a deficiency of MIRL means:
    • Red cells in your body are very prone to lysis because the Membrane attack complex (MAC) cannot be inhibit
    • No protein interferes with the binding of your complement 8 to complement 9
    • Patients with CD59 deficiency will show PNH-like symptoms such as:
      • Hemolysis
      • Strokes
      • Neuropathy
  • Antibody: Anti-CD59.1 (IgG)-in-Patients

Augustine System

  • ISBT Symbol: AUG
  • ISBT Number: 036
  • Responsible Gene: SLC29A1 gene
    • Gene: Equilibrative nucleoside transporter 2 (ENT2)
  • Antigen: Composed of the following
    • AUG1, AUG2 (formerly Ata) which contains Fully developed at birth Resistant to ficin and papain, DTT, and glycine-acid EDTA
      • AUG3 (Newly discovered ANTIGEN)
      • Antibody: Includes the following
      • Anti-Ata (IgG): Which contains
  • Causes severe HTR and mild HDFN (1 case)
    • Anti-AUG3: Severely Causes HDFN
  • Phenotyping antigen: High prevalence, Augustine, and low prevalence
    • High Prevalence
      • AUG2
  • Low Prevalence
    • AUG1
    • AUG3

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