BB common Ag

Lewis Alleles (2)

• Le^a

• Le^b

Lewis Alleles location

Chromosome 19

Lewis sets of Alleles (3)

• Lele

• Sese

• Hh

FUT enzyme adds fucose to GLCNAC makes _____

Le^a

Secretor gene makes _____

Le^b

Lewis bombay genotype (hh) cannot express _______ phenotype

Le^b

Interpretation of Le Se H (RBC phenotype, secretion)

• Le (a-b+)

• Secretes A, B, H, Le in fluid

Interpretation of Le se H (RBC phenotype, secretion)

• Le (a+b-)

• only Le^a

Interpretation of Le Se hh (RBC phenotype, secretion)

• O_para Le (a-b+)

• Secretes A, B (depends on ABO type) and Le^b

Interpretation of Le se hh (RBC phenotype, secretion)

• O_h Le(a+b-)

• NONE

Interpretation of lele Se H (RBC phenotype, secretion)

• Le (a-b-); Le&Se = Le^b+

• Secretes A, B, H substances

Interpretation of lele se H (RBC phenotype, secretion)

• Le (a-b-); Le&Se = Le^b+

• NONE

Lewis Ag secretion is _______ and Ag plasma is ______

• glycoprotein

• glycolipid

Which group (people) has lack of Lewis Ag expression? (2)

• Pregnant women

• Baby (Cordblood RBC)

How long does it take for the Lewis group to be fully expressed?

2-3 years

Which group of people temporarily shows Le (a+b+)?

Children

Is Lewis Ag bound to RBC (completely/passively)?

Passively

Lewis Ab (type, significance)

• Naturally occurring IgM

• not significant

22% of African American have Lewis Ag like …

Le (a-b-)

Which disease attaches to Le^b? (2)

• H.pylori

• Norwalk virus

which Lewis Ag group is at risk for Candida and E.coli infection?

Le (a-b-)

What Ag is found on 98% of RBCs of P Ag group?

LKE

Anti-P₁ (type, significance)

• Naturally occurring IgM

• not significant

Auto anti-P (type, significance, rxn name, characteristics)

• Paroxysmal Cold Hemoglobinuria - IgG

• Significant

• Donath-Landsteiner biphasic hemolysin

• Binds at cold temps, hemolyzes when warmed

Which Ag in P group is the receptor of parvovirus B19

P antigen

I Ag is ______ chain structure and i Ag is ______ chain structure

• branched

• straight

I Ag is expressed in ____ and i Ag is expressed in ______

• all adult

• neonates (cordblood)

How long does it take for i Ag o develop to I Ag?

18 months

Anti I (type, significance)

• Naturally occurring IgM

• Not significant

Cold auto Anti-I and Anti-i with medical conditions (2)

• Mycoplasma pneumoniae

• Infectious mononucleosis/EBV

How does M.pneumoniae make auto Ab and what happens (2)

• The disease has I-like Ag and produces Ab that turns against its own I Ag

• As infection resolves, patient may experience acute hemolysis

Are I & i Ag high prevalent?

Yes

Mn (Allele location, Ab dosage effect, Ab type, agglutinin, significance, proteolytic enzyme effect)

• Glycophorin A (GPA)

• Shows dosage

• IgM

• Cold agglutinin

• Not significant

• destroys M&N Ags

Ss (Allele location, Ab dosage effect, Ab type, agglutinin, significance, proteolytic enzyme effect)

• Glycophorin B (GPB)

• Shows minimum or lower dosage

• IgG

• Warm agglutinin

• Significant (reacts at 37C and AHG test)

• Less likely degrade S&s Ags

Where is Uag located on?

GPB close to RBCs

What is MNS system null phenotype look like, and what Ag do they have?

• M^kM^k

• NEGATIVE FOR M, N, S, s, U, En^a Wr(a-b-) Ag

Which phenotype is En^a?

Partial or complete deletion of GPA, which is a high-frequency Ag.

Which disease is associated with Auto-anti-U and Auto-anti-En^a?

Warm autoimmune hemolytic anemia

Which population has Anti-U and what does it means for RBC phenotype?

• 1% African population

• S-s-U- (U Ag is only present when either S or s Ag present)

Kell Allele Location

Chromosome 7

Kell Low frequency (3)

• K

• Js^a

• Kp^a

Kell High frequency (3)

• k

• Js^b

• Kp^b

Kell Ab (type, significant)

• IgG

• Causes HDFN and reacts in the AHG test

What is the second most immunogenic IgG after D Ag?

Kell Ag

What reagent destroys Kell Ag?

Thiol reagent

How’s Kell associated with HDFN and fatal to the newborn?

Suppression of erythropoiesis due to destruction of erythroid precursor cells

What is K_x named and what is it?

• McLeod phenotype (no Kx)

• X-linked recessive; lacks the Kell system

What is the significance of the McLeod phenotype? (2)

• Hemolytic anemia with acanthocytes

• Myopathy, cognitive impairment (muscle & nerve disorder)

Kell null phenotype

All Kell Ag decreased but Kx increased and may develop anti-Ku (it’s not McLeod)

Duffy Allele location

Chromosome 1

Which population have Fy(a-b-) and what disease is associated?

• 68% African American

• Protection against P.vivax (malaria)

Which Ag is destroyed by common proteolytic enzymes in the Duffy system?

Fy^a & Fy^b

What is GATA?

A mutation in Fy^b is silent in RBCs but appears in other tissues, so Anti-Fy^b is not produced.

Duffy Ab (type, significance)

• IgG

• Significant

Kidd High frequency (3)

• Jk^a

• JK^b

• Jk3

Kidd Ab (type, significant, dosage effect, characteristic)

• IgG but often with IgM and binds C’

• Significant

• Weak dosage on heterozygous Kidd

• Anti-Jk^a has smoking plasma, associated with delayed HTR.

Which Ab titer quickly declines in vivo?

Anti-Kidd (JK)

JKnull phenotype resist cell lysis in ____, a good and quick indication of identifying this particular phenotype

Urea

Which Kidd Ag group is common in African American population?

JK^a

Which Ag group in Lutheran is low frequency, and which one is high frequency?

• Low: Lu^a

• High: Lu^b

Lutheran allele location

Chromosome 19

Lutheran Ab (type, significance, characteristic)

• Lu^a: IgM Lu^b: IgG

• Clinically significant (mild HDFN)

• Loose and mixed filed reactivity in a test tube (different Ab type)

Lutheran Ag (development, enzymes, type)

• Poorly developed Ag at birth (mild HDFN)

• Resistant to ficin and papain, but destroyed by trypsin and α-chymotrypsin

• Glycoprotein on placental tissue

Three types of Lu(a-b-) phenotype

• Dominant: In(Lu) - inhibitor of Lutheran with lost function due to Lmutation in KLF1

• Recessive: silent alleles of LuLu, truly lacks all Lu antigens

• Recessive x-linked: All effected Lu(a-b-) male

List of Dosing Effect (4)

• MN

• Ss

• Duffy

• Kidd