OTHER MAJOR BLOOD GROUP SYSTEM Flashcards
Lewis antigens are not intrinsic to RBCs but are produced by ____ and coded by the production of ____ enzyme.
tissues, fucosyltransferase
The genes coding for fucosyltransferase enzymes in the Lewis system.
FUT1 (H gene), FUT2 (Se gene), FUT3 (Le gene)
The glycosphingolipid type associated with Lewis antigens, adsorbed onto the RBC membrane.
Type 1 glycosphingolipids
The two antigens of the Lewis system.
Le^a and Le^b
The chromosome where the Lewis gene (Le) is located.
Chromosome 19
Le(a-b-) is commonly found in these conditions.
Cord blood, cancer, pregnant women
Phenotype of non-secretors with Lewis gene present.
Le(a+b-)
Phenotype of secretors with Lewis gene present.
Le(a-b+)
The function of the Lewis positive gene (Le).
Converts precursor material to Le^a substance
The result of inheriting Lewis negative genes (lele).
Le(a-b-) phenotype
The phenotype of a person with at least one Le gene and one Se gene.
Le^b positive
The phenotype of a person with at least one Le gene and sese genes.
Le(a+b-)
The gene controlling ABH secretion but not Lewis secretion.
Se gene
Reaction characteristics of anti-Le^bH.
Reacts best when both Le^b and H antigens are present
Reaction characteristics of anti-Le^6L.
Recognizes any Le^b antigen regardless of ABO type
Characteristics of Lewis antibodies.
Naturally occurring, generally IgM, neutralizable by plasma
Role of Le^a^b in pathogen interactions.
Associated with H. pylori and Norwalk virus receptor
Marker associated with Reed-Sternberg cells in Hodgkin’s lymphoma.
Le^x
Clinical implications of Le(a-b-) phenotype.
Increased renal graft survival, heart disease risk, susceptibility to Candida and uropathogenic E. coli
The most common Lewis antibody.
“Anti-Le^a” - seen in nonsecretors
Le^a+b+ phenotype is rare in which population?
East Asian descent
Genes involved in Le^a+b+ phenotype
Lewis (FUT3) and weak secretor (FUT2)
Characteristic of Le^a+b+ phenotype
Both Le^a and Le^b antigens on RBCs
Effect of weak secretor status on Le^a+b+ phenotype
Reduced conversion of Le^a to Le^b
Antigens in the P blood group system
P, P1, Px, LKE
Biochemical relation of P1PK (003), Globoside (028), and Related Antigens
P1PK - P1,PK, Globoside - P, Px, LKE
Antigens formed in the P blood group system
P1, Px, P
Receptor for Parvovirus B19
P antigen
Prevalence of P1 antigen on red cells
Present in 79% of the population
Individuals lacking P1 antigen are called
P2
Phenotype of individuals lacking P1, PK, and P antigens
P null or p
Time of P1 antigen presence on fetal red cells
Present as early as 12 weeks, weakens with gestational age
Storage effect on P1 antigen
Deteriorates rapidly
Substances containing Pl antigen
Hydatid cyst fluid, earthworm (Lumbricoides terestris), Ascaris suum
Anti-P1 antibodies are found in which sources
Liver flukes, tapeworms, earthworms, pigeons, turtledoves
Anti-P1 antibody type
Naturally occurring IgM
Anti-P, P1Pk antibody is associated with which condition
Spontaneous abortions
Original name of Anti-P, P1Pk antibody
Anti-Tja
Alloanti-P antibody characteristics
Rare, hemolytic, reacts over a wide thermal range
Autoanti-P is found in which disease
Paroxysmal Cold Hemoglobinuria (PCH)
Test for Autoanti-P
Donath-Landsteiner Test(expose blood at 4°C, then 37°C, check for hemolysis)
Anti-Pk antibody is isolated from which cells
P1 cells
Anti-Pk antibody is reported in which conditions
Biliary cirrhosis, autoimmune hemolytic anemia
Luke (LKE) antigen is a marker for which cells
Embryonic and mesenchymal stem cells
Luke (LKE) antigen disease associations
Hemolytic uremic syndrome (HUS), HIV protection, apoptosis in B cells, Burkitt lymphoma, lymphoblastic leukemia
I antigen can also be found on which cells
WBC, platelets, cord blood cells
Prevalence of I antigen
High in adults (trace i antigen in adults)
Transition of i to I antigen
i decreases, I increases by 1.5 to 2 years
Rare phenotype of I antigen
i adult or I negative
Anti-I association
Cold hemagglutinin disease, M. pneumoniae
Anti-i association
Infectious mononucleosis
I and i antigens sources
Plasma, serum, saliva, human milk, amniotic fluid, urine, ovarian cyst fluid
Conditions with increased I antigen
Acute leukemia, (hypoplastic anemia i antigen), megaloblastic anemia, sideroblastic anemia, thalassemia, sickle cell disease, PNH, chronic hemolytic anemia, HEMPAS
MNSs system location
Chromosome 4
Effect of enzymes on MNSs antigens
Destroyed by enzymes
Antigen type of MNSs system
Red cell antigens, not in secretions
Anti-M antibody type
Naturally occurring IgM
Optimal pH for Anti-M reaction
pH 6.5
Anti-Nf antibody associated condition
Renal patients, IgM
Anti-S/Anti-s antibody reaction temperature
37°C, antiglobulin test phase
MN antigens location
Glycoprotein A
M and N antigen amino acid differences
M: Serine at 1, Glycine at 5; N: Leucine at 1, Glutamate at 5
Development of MN antigens
Well developed at birth, enzyme-sensitive
Ss antigens location
Glycophorin B
Critical amino acid for Ss antigen expression
Methionine at position 29 for S, Threonine for s
Resistance of Ss antigens to enzymes
Less easily degraded by enzymes
U antigen presence
Always present with S or s inheritance, resistant to ficin
SsU phenotype genetic basis
GYPB deletion
En(a) phenotype genetic basis
GYPA deletion
MNSs null phenotype
GYPA + GYPB - MkMk
Parasite receptor function of M and N
Receptor for Plasmodium falciparum
Resistance to malaria in M-N- phenotype
Resistant to P. falciparum
Order of immunogenicity among ABO, D, and Kell
ABO > D > Kell
Kell system chromosome location
Chromosome 7
First blood group system discovered after antiglobulin testing
Kell
Antithetical antigens in Kell system
K-Kell, k-Cellano; Kpa-Penney, Kpb-Routenberg; Jsa-Sutter, Jsb-Matthews
Kell antigen dependency
Dependent on the presence of Kx protein
Kx antigen system characteristic
Kx is the only antigen in the Kx system
K0 phenotype characteristic
Null phenotype in the Kell system
Most common antibody in Kell system (excluding ABO and Rh)
Anti-K
Bacteria associated with anti-K production
E. coli O125:B15
McLeod phenotype characteristic
Absence of Kx, Km; depression of all Kell antigens, associated with GD defect
Blood abnormalities in McLeod phenotype
Presence of acanthocytes
Gender most affected by McLeod phenotype
Male
Source of Kell antigen identification
Serum of Mrs. Keller
Duffy system similarity to other blood groups
Similar to MNS, Sirbon, Mulligans, and P. knowlesi systems
Duffy antigens
Fya and Fyb
Effect of enzymes on Duffy antigens
Destroyed by common proteolytic enzymes (ficin and papain)
Duffy antigen expression during development
Identified on fetal red cells as early as 6 weeks gestation and expressed at birth
Receptor role of Duffy antigens
Receptors for Plasmodium vivax and Plasmodium knowlesi
Malaria resistance associated with Duffy phenotype
Fya-b- is resistant to Plasmodium vivax and P. knowlesi
Null phenotype in Duffy system
Fy null (Fya-b-) confers resistance to Plasmodium vivax and P. knowlesi
Function of Fya-b-
Acts as a chemokine receptor
Duffy antigen polymorphism amino acid positions
Fya has glycine, Fyb has aspartic acid at position 42
Duffy system characteristic regarding dosage
Demonstrates dosage effect
Type of antibodies for Anti-Fya and Anti-Fyb
Usually IgG
Reaction phase of Anti-Fya and Anti-Fyb
Best at antiglobulin phase
Effect of low ionic strength medium on Anti-Fya and Anti-Fyb
Activity is enhanced
Reaction of Anti-Fya and Anti-Fyb with enzyme-treated red cells
No reaction
Clinical significance of Anti-Fya and Anti-Fyb
Associated with hemolytic transfusion reactions; hemolysis is usually mild
Anti-Fya and HDFN severity
Often mild, occasionally severe
Prevalence of Anti-Fya vs Anti-Fyb
Anti-Fya is more commonly observed
Function of Kidd antigens
RBC urea transporter
Kidd antigens
Jka, Jkb, Jk3
Development of Kidd antigens
Well-developed at birth; Jka detected at 11 weeks, Jkb at 7 weeks
Effect of enzymes on Kidd antigens
Enhanced by enzymes
Silent allele in Kidd system
Jk allele; common in Polynesians, Filipinos, and Chinese
Phenotype resulting from JkJk genotype
Jk(a-b-)
Practical test for Jk(a-b-) phenotype
Resistance to 2M urea
Clinical significance of anti-Jka and anti-Jkb
Common cause of delayed hemolytic transfusion reactions (HTR)
Characteristic of anti-Jka and anti-Jkb in vitro
Titer quickly declines
Notorious reputation of anti-Jka and anti-Jkb
Difficult to detect in the blood bank
Clinical significance of anti-Jk3
Associated with severe immediate and delayed HTR and mild HDFN
Lutheran antigens
Lua, Lub
Development of Lutheran antigens
Poorly developed at birth; reach adult levels by age 15
Incidence of most Lutheran antigens
High incidence
Discovery of Lutheran blood group
Named due to a labeling error of donor ‘Lutteran’
Antibody classes in anti-Lua
IgG, IgM, IgA
Antibody class in anti-Lub
IgG (immune antibody)
Effect of enzymes on Lutheran antigens
Affected by enzymes
ISBT number for Lutheran system
ISBT 005
Immunoglobulin classes of Anti-Lua
IgM and IgG
Optimal temperature for Anti-Lua agglutination
Room temperature
Clinical significance of Anti-Lua
No clinical significance in transfusion; mild HDFN reported
Characteristic pattern of Anti-Lua agglutination
Mixed-field pattern
Immunoglobulin class of Anti-Lub
IgG
Phase of agglutination for most Anti-Lub reactions
Antiglobulin phase
Clinical significance of Anti-Lub
Associated with transfusion reactions and mild HDFN
Why is Anti-Lub rare?
Due to the high incidence of Lub antigen
Mixed-field agglutination in Anti-Lub
Some examples show mixed-field agglutination
