OTHER BLOOD GROUP SYSTEMS Flashcards
BGS of 001?
ABO, Ang
BGS of 002?
MNS, Mens
BGS of 003?
P, Po (ni)
BGS of 004?
Rh, Rhea
BGS of 007?
Lewis, Lang
BGS of 005?
Lutheran, Lumabas
BGS of 006?
Kell, Kaya
When some of these blood group systems are present, RBCs are in normal shape.
STRUCTURAL RELATIONSHIP TO RED CELL
BGS of 009?
Kidd, Kita (ni)
BGS of 008?
Duffy, Di
- Transferases-transfer enzyme
GLYCOSYLTRANSFERASES
BGS of 010?
Diego, Diego
What poikilocyte is associated with Gerbich Null blood group system?
- Elliptocytes
Enzymes responsible for the initiation and elongation of glycan chains on mucins as they transfer activated sugar residues to the proper acceptor.
GLYCOSYLTRANSFERASES
TRANSPORT PROTEINS BGS examples?
Rh, Kidd, Diego, Colton, and Kx blood group systems
STRUCTURAL RELATIONSHIP TO RED CELL BGS example?
MNS, Diego, and Gerbich blood group systems
GLYCOSYLTRANSFERASES BGS example?
Some are carbohydrates (ABO), P1PK, Lewis, and H blood groups systems
What poikilocyte is associated with RhNull blood group system?
stomatocytes
- Not clinically significant because IgG in nature are more clinically significant because they react at 37℃ (body temp) True or False?
TRUE
- The Secretor (Se, FUT2) gene is located on
chromosome 19
Under COMPLEMENT PATHWAY MOLECULES what component is associated in accelerating factor?
- Cromer
COMPLEMENT PATHWAY MOLECULES components?
- Chido Rodgers- associated with C4b
- Cromer-associated in accelerating factor
- Knops blood group systems
Under COMPLEMENT PATHWAY MOLECULES what component is associated with C4b?
- Chido Rodgers
ADHESION MOLECULES BGS examples?
Lutheran, Xg, Lansteiner-Wiener, and Indian blood group systems (predominant blood group in ARAB population)
- The Lewis (Le, FUT3) gene is located on
chromosome 19
MNS, Duffy, P, Lewis, and Cromer blood group systems
Microbial Receptors
BIOLOGIC RECEPTORS BGS examples?
Duffy, Knops, and Indian blood group system
Lewis blood group are susceptible of having (?) which causes ulcer
Helicobacter pylori
- Named after one of the first individuals to make the antibody (?), reported by Mourant in 1946.
Lewis Blood Group System
2 alleles at the Lewis locus
L, e, l, e
FUT1 what gene?
H
FUT2 what gene?
Se
- Must be present for a precursor substance to be converted into Lea
Le gene Lea →
If ever we have the secretor gene, we cannot secrete or manifest ABO blood group in the body fluids TRUE or FALSE?
False
2 alleles at the secretor locus
S, e, s, e
- Must also be present for the conversion of Leb
Se gene Leb →
- These Le genes will influence How many (?) phenotypes, and will result to the interaction of the two genes
4
- If no Le gene is present, and only le is present then we will have no formation of Lea . same as true in Se gene TRUE or FALSE
True
a term used to describe a pair and occasionally more than 1 pair of antigens that are coded by different alleles of a single gene
antithetical
Lewis Blood Group is?
antithetical
- Are not expressed on cord RBCs and are often diminished on the mother’s RBCs during pregnancy.
LEWIS ANTIGENS: Lea and Leb
are resistant to treatment with the enzymes that has ficin and papain, DTT (Dithiothratol) and glycine acid EDTA
Lewis antigens
LEWIS ANTIGENS: Lea and Leb can be found in?
Found on lymphocytes and platelets and on other tissues such as pancreas, stomach, intestine, skeletal muscle, renal cortex, and adrenal glands
- found in saliva as glycoproteins
- Soluble antigens
Lewis antigen (Lea and Leb ) are not intrinsic to RBCs but are on (?) that are passively adsorbed onto the RBC
Type 1 glycosphingolipids
Gram negative bacteria that is associated with gastritis, peptic ulcer, gastric carcinoma, norwalk virus
Helicobacter pylori
The Leb antigen is the receptor for what bacteria?
Helicobacter pylori
- Are IgM and have no clinical significance.
- Have not been implicated in HDFN because the antibodies do not cross the placenta, and the antigens are not well developed at birth.
LEWIS ANTIBODIES: Anti-Lea and Anti-Leb
The most commonly encountered of the Lewis antibodies is?
Anti-Le^a
One of the function of IgG is that is efficient in binding the complement TRUE or FALSE?
False, it should be IgM
Le (a-b-) what is the white and blacks?
White: 6, Black: 22
Le (a+b-) what is the white and blacks?
White: 22, Black: 23
Le (a-b+) what is the white and blacks?
White: 72, Black: 55
How many percent are non-secretors
- 20%
Le (a+b+) what is the white and blacks?
White: rare, Black: rare
How many percent of the white population are secretors
78 to 80%
In terms of Le(a-b-) individuals
o (?) are secretors
o (?) are non-secretors
80%; 20%
arises from the inheritance of Le, Se and H gene.
Le (a-b+) red cell phenotype
Lewis antigens found in the SECRETIONS are
glycoproteins
Lewis antigens found in the PLASMA are
glycolipids
from newborn infant’s phenotype as Le (a-b-).
Cord blood and RBCs
comprised the P, P1 and Pk antigens and later, Luke (LKE).
P BGS
is assigned to the Globoside blood group system (028, GLOB).
P
are not detectable in plasma until about 10 days after birth.
Lewis glycolipids
Red cells adsorb only (?) and not (?), onto the membrane.
glycolipids,glycoproteins
- A person can be a nonsecretor (sese) and still secrete Lea into the body fluids. TRUE or FALSE?
True
P gene located at chromosome
chromosome 3 q26.1
P BGSCommon precursor?
Lactosylceramide (Gb2)
are assigned to the P1Pk Blood Group system (003, P1Pk)
P1 and Pk
are assigned to the Globoside Collection (029, GLOB).
LKE and PX2
P1PK gene is located at chromosome
chromosome 22 q11.2
is also used for the production of ABO.
- Paragloboside
Under P BGS: RBCs, lymphocytes, granulocytes, and monocytes
P1, P, Pk
plasma as glycosphingolipids and as glycoproteins in hydatid cyst fluid.
P and Pk
Under P BGS:platelets, epithelial cells, and fibroblasts.
P
Parasite that produces cyst
Echinococcus granulosus.
o ➔ Poorly expressed at birth and may take up to 7 years to be fully expressed.
o ➔ It deteriorates rapidly on storage
o ➔ Blacks have stronger expressions of P1 than whites.
P Antigen
2 categories of P Ab
- Clinically insignificant
- Potently hemolytic
➔ Common, naturally occurring IgM antibody in the sera of P- (negative) individuals.
➔ Typically weak, cold reactive saline agglutinin optimally reactive at 4 degrees Celsius (IgM) and not seen in routine testing.
Anti-P1
Anti-P1 is associated in?
parasitic infections.
➔ Infected with Echinococcus granulosus tapeworms led to the identification of P1 and Pk substance in hydatid cyst fluid
Anti-P1 in P1 individuals
have also been found in patients with fascioliasis (bovine liver fluke disease) and in bird handlers.
Anti-P1 in P1 individuals
is a parasitic infection typically caused by Fasciola hepatica
Fascioliasis
Anti-P1 in P1 individuals associated to?
tumor hydatid mole.
➔ It has the potential to cause severe HTR and HDFN.
Anti-PP1Pk (example of potent hemolysis)
➔ Originally called anti-Tja.
➔ First described in the serum of Mrs. Jay, a p individual with adenocarcinoma of the stomach.
Anti-PP1Pk (example of potent hemolysis)
Anti-PP1Pk (example of potent hemolysis) is associated in?
➔ It is also associated with an increased incidence of spontaneous abortions in early pregnancy
Autoanti-P associated with PCH (Paroxysmal Cold Hemoglobinuria) associated in?
➔ Associated with the cold reactive IgG autoantibody in patients with PCH
➔ Rarely seen but is very significant in transfusion.
➔ IgG class anti-P may occur and has been associated with habitual early abortion.
Allo Anti-P (antibody being formed against different individual but same specie)
can react both in a cold and warm temperature.
- Biphasic
- Antibody binds to RBCs in the cold.
- Via complement activation, the coated RBCs lyse as they are warmed to 37 degrees Celsius
IgG autoantibody in PCH-biphasic hemolysin:
➔ Demonstrated only by the Donath-Landsteiner Test. ➔ Tube will be exposed to cold temperature then warm.
➔ Upon warming, we can observe lysis aside from agglutination.
Autoanti-P associated with PCH (Paroxysmal Cold Hemoglobinuria)
→ Described by Tippett and colleagues in 1965 in the serum of a patient with Hodgkin’s Lymphoma.
Luke (LKE) Antigen
Luke (LKE) Antigen 3 phenotypes?
● 80% tested Luke+
● 14% Luke (w)
● 2% Luke-
- Lewis Blood group system are also seen in secretors. TRUE or FALSE?
TRue
Disease-associated in P BGS:
Parasitic infections
- Echinococcus granulosus
- fasciola hepatica/ fascioliasis
Anti-P1
- ISBT System Symbol:
I
Disease-associated in P BGS: Early abortions
● Anti-PP1Pk or Allo anti-P
Disease-associated in P BGS: : (PCH) paroxysmal cold hemoglobinuria
● Autoanti-P
All individuals with the p and Pk phenotype are
Luke
Disease-associated in P BGS: provides some protection against HIV infection of peripheral blood mononuclear cells
● Pk
Disease-associated in P BGS: receptor for shiga toxins, which cause shigella dysentery and E.coli- associated HUS
● The Pk antigen
Disease-associated in P BGS: receptors for P-fimbriated uropathogenic E.coli (Causes UTI).
● The P system antigens
- ISBT System Number
027
Disease-associated in P BGS: is the receptor of human parvovirus B19 - 5th disease of childhood with slapped cheek appearance
● P
→ Rare
→ Slightly more common in Japan, North Sweden, and in an Amish group in Ohio
p Phenotype
- “I” and “i” have also been found in the
the plasma and serum of adults and newborns and in saliva, human milk, amniotic fluid, urine, and ovarian cyst fluid.
ISBT Clinical Significance
NO
I for (?)
individulaity
antigens are found on the membranes of leukocytes and platelets in addition to RBCs.
“I” and “i”
Infants RBCs are rich in “?”: “?” is almost undetectable.
i; I
Patients with this condition will often develop strong agglutination with “i” or “I” specificity and can experience a transient episode of acute and abrupt hemolysis as the infection resolves
Mycoplasma pneumonia infections (walking pneumonia).
During the first (?)months of life, the quantity of “i” slowly decreases as “I” increases until adult proportions are reached.
18 months
- Found in the serum of many normal healthy individuals and is benign- it is not associated with in vivo RBC destruction (intravascular hemolysis)
Autoanti-I
- It is a common autoantibody that can be found in virtually all sera.
- Testing at 4°C and/ or against enzyme treated RBCs may be required to detect the reactivity.
- It is not associated with HDFN because the antibody is IgM, and the I antigen is poorly expressed on infant RBCs.
Anti-I
Weak, naturally occurring, saling reactive IgM agglutinin
Autoanti-I
Pathogenic autoanti-I associated with?
Cold Agglutinin Disease/CAD.
- When peripheral circulation cools in response to low ambient temperatures,these antibodies attach in vivo and cause agglutination and peripheral vascular occlusion (acrocyanosis) or hemolytic anemia
Pathogenic autoanti-I
- The production of autoanti-I may be stimulated by microorganisms carrying (?) like antigen on their surface.
10
Autoanti-I is associated with?
Mycoplasma pneumonia infections (walking pneumonia).
of IgM and IgG anti-l reacting preferentially at 37°C have also been found in patients with (?) with a special association with Hodgkin’s disease
WAIHA (Warm Autoimmune Hemolytic Anemia)
- Typically reacts with adult and cord RBCs equally well at room temperature and at 4°C.
Pathogenic anti-I
- Adult i – also known as
HEMPAS (Hereditary Erythroblastic Multinuclearity with Positive Acidify Serum test)
- Exists as an IgM or IgG antibody in the serum of most individuals with the adult “i” phenotype
Alloanti-I
have also been described and have been associated with HDFN.
- IgG Anti-i
- Reacts strongly with cord RBCs, weakly with normal adult RBCs and most weakly with adult i RBCs
I^t Antigen and Antibody
never described.
- Alloanti-i:
Potent examples are associated with infectious mononucleosis (Epstein Barr virus infections) and some lymphoproliferative disorders. Associated with hempas antigen. hereditary erythroblastic multinuclearity with positive acidified serum lysis test
- Autoanti-i
