OTHER MAJOR BLOOD GROUPS

• Autosomal chromosomes

• Mendelian inheritance

Most blood groups are located on the ____ and demonstrate straightforward ____

Determine which of the ff. are the:

1. Allele

2. Antigen

3. Phenotype

Genotype

A probable interpretation as to which genes the individual carries in order to see the phenotype.

• Carbohydrate (sugars) attached on glycoprotein or glycolipids

• Amino acids on a protein

Blood group antigens are defined by the presence of?

• 028

• 006

• 008

• 009

• 001

• 002

• 004

• 003

• 007

• 005

State the ISBT number.

• Globoside

• Kell

• Duffy

• Kidd

• ABO

• MNS

• Rh

• P

• Lewis

• Lutheran

• Enzymatic

• Structural proteins

• Transport-water-soluble molecules

The Physiologic Functions related to RBC Membranes.

• __ activities

• ____ - maintain RBC shape/ mechanical deformability

• _____ across lipid bilayer for intake of nutrients and excretion of waste products

• Complement

• Adherence

• Microbial receptors

• Duffy antigens - Fya and Fyb

The Physiologic Functions related to RBC Membranes.

• ___ pathway RBC membrane interactions

• Cell ___ of cells

• RBC antigens as ___ for infection by microorganisms

• These antigens serve as attachment sites for malarial parasites

• ABO

• P1PK

• Lewis

• H

Their functional role includes glycosyltransferases

• MNS

• Diego

• Gerbich

Their functional role includes structural relationship to red cell

• Rh

• Kidd

• Diego

• Colton

• Kx

Their functional role includes transport proteins

• Chido/ Rodgers

• Cromer

• Knops

They function as complement pathway molecules

• Lutheran

• Xg

• Landsteiner-Wiener

• Indian

They function as adhesion molecules

• MNS

• Duffy

• P

• Lewis

• Cromer

They function as microbial receptors

• Duffy

• Knops

• Indian

They function as biologic receptors

• Landsteiner and Levine

• Walsh and Montgomery

• Greenwalt and colleagues

• Who discovered M and N antigens?

• Who discovered S antigen?

• Who discovered that U-RBCs are also S-s, resulting to the inclusion of U into the system?

• MNS

• 002

• No

• IgM

• Cold temperature

• Room Temperature, Anti Human Globulin

• No effect

MNS BLOOD GROUP SYSTEM.

M and N antigens

• ISBT symbol

• ISBT number

• Clinical Significance

• Antibody class

• Optimal temperature

• Reaction Phase

• Effect of enzyme

Glycophorins

These are glycoproteins that are the outer layer of the RBC membrane and it carries many blood group antigens.

Glycophorin A (sialoglycoprotein)

This is the major RBC sialic acid-rich glycoprotein

Glycophorin A

MN antigen is found on?

• M - position 1: Serine and 5: Glycine

• N - position 1: Leucine and 5: Glutamic acid

MN Antigen

• What are their respective positions?

• What are their respective amino acids?

Enzyme treatment - potentiators

• Ficin

• Papain

• Bromelin

• Trypsin

• Pronase

• ZZAP (DTT, papain, ficin)

MN antigens are easily destroyed by?

• DTT alone

• 2-aminoethyliso-thiouronium bromide (AET)

• α-chymotrypsin

• Chloroquine

• Glycine acid EDTA treatment

MN antigens are not affected by what enzymes?

Neuraminidase

It cleaves sialic acid

Anti-M

Most examples are naturally occurring, cold reactive saline agglutinins. It is more common in bacterial infections.

Yes

Is Anti-M affected by antigen dosage?

• IgM (majority); 50-80% IgM

• Does not bind

• None

• 6.5 pH and glucose solutions

ANTI-M.

• Immunoglobulin involved

• Complement binding

• Enzymatic reaction

• Reacts with?

Anti-N(f)

Seen in renal patients who are dialyzed on equipment sterilized with formaldehyde

Anti-Nf

» will attack both N antigen and Formaldehyde

If there is contamination of formaldehyde, what will be the antigen that is produced?

Glycophorin B

Ss antigens are found on?

• Position 29

• S - methionine; s - threonine

Ss antigens

• What are their respective positions?

• What are their respective amino acids?

Inner portion of RBC

» that is why it is only slightly affected by enzyme treatment

» GA is found on the surface, hence it is easily affected.

Glycophorin B is found where?

• IgG

• 37 degrees C

• Antiglobulin test phase

• Fix complement

ANTI-Ss

• Immunoglobulin

• Reactive at what temperature?

• Reactive at what test?

• Complement

• Severe HTR with hemoglobinuria

• HDFN

Anti-Ss is implicated with what conditions?

Anti-S

This antigen in MNS can cause discrepant typing results.

• MNSs Blood Group

• GYPA - 7, GYPB - 5

• GYPA and GYPB are genes seen in?

• How many exons do they have?

Ss antigen → SsU

It is linked with U antigen

• In 1% in American blacks

• 1-35% Africans

• U phenotype is present in all individuals except?

• U-negative cells are found in these individuals.

U antigen

This antigen is present on individuals who lack GPB because of a partial or complete deletion of GYPB.

U variant (Uvar)

Anti-U that react weakly with apparent U- RBCs. They have altered GPB that does not express S or s.

• Ena

• Darnborough and Furuhjelm

• A high-prevalence antigen that reacted with all RBCs except those of the propositi.

• It is described by?

Anti-Ena

An umbrella term for reactivity against various portions of GPA unrelated to M or N.

• Anti-EnaTS

• Anti-EnaFS

• A trypsin-sensitive Ena

• A ficin-sensitive Ena

Mk

A rare silent gene that does not produce M or N antigens and is silent at the Ss locus.

MkMk

It represents a near-complete deletion of both GYPA and GYPB; hence, it is the null phenotype in the MNS system.

Warm-type autoimmune hemolytic anemia

Autoantibodies to U and Ena are associated with what disease?

GPAM

A MNS antigen that serve as the receptor for certain pyelonephritogenic strains of E.coli to enter urinary tract.

Malaria parasite (Plasmodium falciparum)

GPA and GPB (also NeuNAc) are receptors for what organism?

• P1PK

• 003

• No

• IgM

• Cold temperature

• Room temperature

• Elevated

P1 Antigen

• ISBT Symbol

• ISBT number

• Clinical significance

• Antibody class

• Optimal temperature

• Reaction phase

• Effect of enzyme

• Globoside blood group (028)

• Globoside collection (209)

The ff. are originally in P Blood Group but was reassigned. In what blood groups were they transferred?

• P and PX2

• LKE

Landsteiner and Levine

Who introduced P blood group?

Levine and colleagues

Who discovered anti-Tja?

• Anti-P1

• P1

• P2

• p

Determine how the ff. are renamed.

• Anti-P

• P+ phenotype

• P- phenotype

• P null

• P1 and P2

• p, P1k, P2k

• The 2 common phenotypes of P blood group.

• The 3 rare phenotypes.

P1

Determine the phenotype.

• Red cells express P, P1, and Pk antigens

P2

Determine the phenotype.

Lacks P1 antigen but expresses P and Pk antigens

• P2. Because it lacks P1 antigen.

• P1 will not create antibodies because it contains all antigens.

• Which between P1 and P2 creates antibodies?

• Why?

• P, P1, PK

• P, PK

• P1, PK

• PK

• null (did not inherit antigens)

Determine the antigens present.

• P1

• P2

• P1K

• P2K

• p

P1 antigen

• Found on fetal red cells as early as 12weeks, full expression on 7 years but it weakens with gestational age

• Deteriorates rapidly on storage

P1 antigen

It is found on plasma, droppings of pigeons and turtledoves, or egg whites of turtledoves.

Echinococcus granulosus

What parasite produces hydatid cyst?

• Hydatid cyst

• Lumbricoides terrestris (common earthworm)

• Ascaris suum

• Echinococcus granulosus

• Fasciolasis (bovine liver fluke disease)

P1 antigen is found in what parasites?

P1 and Pk

What substances are seen in hydatid cyst fluid?

• Anti-PP1PK

• Anti-Tja (means tumor)

• Mrs. Jay, adenocarcinoma of stomach

• P null

• Other term and its meaning

• Where was it found?

• Spontaneous abortion in early pregnancy

• Severe HTRs and HDFN

Anti-PP1PK is associated with what conditions?

IgG anti-P

What antibody causes conditions in most P individuals?

• Paroxysmal Cold Hemoglobinura (PCH)

• Donath Landsteiner test

• Anti-P is associated with what disease?

• How is it demonstrated?

Biphasic

The antibody activity of Anti-P ___, meaning it attaches to red cells during cold, but lyses them as they warm.

• LKE antigen

• Lewis X

• Anti-It

Antigens seen in Hodgkin’s Lymphoma

Alloanti-P

It is rarely seen in the blood bank but very significant in transfusion because it is hemolytic with a wide thermal range of reactivity.

Luke -

All indivdiuals with p and Pk phenotype are?

PX2

A product of BEGALNT1

P and Pk Antigens / Uroepithelial cells

P system antigens thatserve as receptors for P-fimbriated uropathogenic E. coli

Anti-Pk

Has been reported in the serum of P1 individuals with biliary cirrhosis and autoimmune hemolytic anemia

Pk antigen

A receptor for Shiga toxins, which cause Shigella dysentery and E. coli associated hemolytic uremic syndrome

P / Globoside

Receptor for Human Parvovirus B19

Anti-Lua (Lutheran)

It is found in the serum of a patient with lupus erythematosus

• LU

• 005

• Lub - yes, Lua- no

• Lub - IgG, Lua - IgM

• Warm and Cold

• Lub - AHG, Lua - RT

• Present

LUTHERAN BLOOD GROUP

• ISBT symbol

• ISBT number

• Clinical significance

• Optimal Temperature

• Reaction Phase

• Effect of enzyme

• Cutbush and Chanarin

• Crawford et al.

• They described anti-Lub

• They described Lu (a-b-)

Anti-Lua

• It often goes undetected in routine testing because most reagent RBCs are Lu(a–).

• More likely encountered as an incompatible crossmatch or during an antibody workup.

Anti-Lua

Has loose, mixed-field reactivity in a test tube

Anti-Lub

Has been implicated with shortened survival of transfused cells and post-transfusion jaundice.

Laminin

The Lutheran proteins are multifunctional adhesion molecules that bind ___, notably in sickle cell disease.

• BCAM

• 19

• Lu and Se gene linkage

• Gene present in Lutheran

• Chromosome

• The first example of autosomal linkage described in humans.

In (Lu)

The inhibitor for Lutheran

In (Lu) → Dominant Type Lu(a–b–)

Mutations in the gene for erythroid Krüppel-like factor (EKLF), a transcription factor, were shown to be associated with the?

Dominant Lu(a-b-)

• Weak Lu Antigen

• No Lu Antibody

Recessive Lu(a-b-)

• No Lu antigen

• Presence of Lu Antibody

Recessive type Lu(a-b-)

It demonstrates recessive inheritance, the result of having two rare silent alleles LuLu at the Lutheran locus.

Recessive X-Linked Inhibitor Type

Mutation in the X-linked gene for the major erythroid transcription factor GATA-1 has been shown.

Anti-Lu3

• A rare antibody that reacts with all RBCs except Lu(a–b–).

• It recognizes a common antigen whenever Lua or Lub is present.

Kell Blood Group

The first blood group system discovered after the introduction of antiglobulin testing.

• KEL

• 006

• Yes

• IgG

• Warm temperature

• Anti-Human Globulin

• No effect

KELL BLOOD GROUP SYSTEM.

• ISBT symbol

• ISBT number

• Clinical significance

• Antibody class

• Optimal temperature

• Reaction Phase

• Enzymatic effect

• Kelleher (1946)

• Celano (3 years after KEL 1)

• Who discovered KEL 1 or K?

• Who discovered KEL 2 or k?

K0

Kell null phenotype

Xk protein

The protein associated with Kell Blood Group.

K antigen

It is rated second only to D in immunogenicity.

• Kpb and Jsb

• Kpa, Kpc, and Jsa

• High prevalence antigens in Kell Blood Group

• Low prevalence antigens

Bacterial infections (E.coli O125:B15)

IgM Anti-K are rare and associated with what disease?

• Anti-K

• Anti-K titers at 64

• Associated with severe HTRs (extravascular via the macrophages in the spleen) and severe HDFN.

• Seen in stillbirth

Anti-Kpa

Kell antibody that is naturally occurring.