Bb lec Flashcards
Found and soluble in plasma and saliva
Lewis
“Plasma
antigen”
Lewis
Expression of the Lewis antigens are influenced by
Hh and Sese antigens
is weak during pregnancy - Mothers
who are Le(a-b+), may be Le(a-b-)
Lewis
When was Lewis first discovered?
1946
Agglutinated RBCs from about 25% of English people
Lewis
When was Anti-Leb discovered?
1948
Le genes (Le/FUT3) are located at
short arm of chromosome 19p13.3
Le gene is linked to
Se and H genes
The gene does not actually code for the production of Lewis antigens but rather produce specific
L-fucosyltransferase to type 1 precursor substance
The system depends on 3 genes to produce the Lewis antigen
H, Se, Le genes
There are two alleles at the Lewis locus:
Le and le genes
There are two alleles at the secretor locus:
Se and se genes
Development of Lewis antigen structure begins in
first week after birth and may continue for six years
Also found on lymphocytes and platelets
Lewis antigens
-Lea substance is secreted regardless of secretor status
-Le gene adds “L-fucose” to the N- acetylglucosamine of the type 1 precursor substance to form the Lea antigen
Le(a+b-)
Le gene adds “L-fucose” to the N- acetylglucosamine, and H gene adds “L-fucose” to galactose of the precursor substance forming the Leb antigen
Le(a-b+)
Lewis antigens are resistant to treatment by enzymes
(Ficin and Papain), Dithiothreitol, and Glycine-acid EDTA
IgM, Naturally occuring
Lewis antibodies
Reacts at immediate spin
Lewis antibodies
Binds complement, and triggers in-vitro hemolysis
Lewis antibodies
(History) The antibody Anti-K was first identified in the serum of Mrs. Kelleher. It reacted with the RBCs of her newborn infant, her older daughter, and her husband. Discovery by Robin Coombs
1946
Kpa antigen and the null phenotype (Ko) were first described in
1957
Jsa were first described, named after the first producer
John Sutter
The low-incidence antigen of Kell
K24
The Kel gene is found on chromosome
7q33/34
The different Kell antigens are due to
single base mutations that results to amino acid substitutions
Member of Neprilysin family
Kell
The Kell antigens are located on a
type II glycoprotein with 731 amino acids
The glycoprotein is linked with XK protein by a disulfide bond at
Cys72
The glycoprotein is linked with XK glycoprotein by a disulfide bond at
Cys347
Found only on RBCs
Kell antigens
Appears on fetal red cells earlier than Rh proteins
Kell antigens
Kell glycoprotein has been characterized as a _________which is central to zinc binding and catalytic activity
Zinc endopeptidase
Very immunogenic (second only to D)
K antigen
Detected to as early as 7 weeks
k
Gene encoding for the antigen is associated with suppression of other Kell antigens
Kpa
Can be detected on fetal RBCs as early as 10 weeks gestation and is fully developed at birth
K
Found in 20% of blacks
Jsa
A patient’s red cells lacks the entire Kell glycoprotein, therefore, no Kell antigens
Kell null phenotype
Who identified Kell null phenotype
Bruce Chown, Marion Lewis, and Kiroko Kaita in 1957
The most encountered antibody next to ABO and Rh
Anti-K
Associated with severe HTRs and severe HDFN
Anti-K
Patients should receive antigen-negative blood
Anti-K
Are not commonly detected because individuals who lack these high-incidence antigens are scarce
Anti-Kpa, Anti-Kpb, Anti-Kpc, Anti-Jsa, Anti-Jsb
This antigens are only found in asians
Kpc
Found in erythroid tissues, brain, lymphoid organs, heart, skeletal muscle
Kx
The XK gene that encodes for the Xk protein is independent to KEL gene and found in the
short arm of chromosome Xp21.1
Kx antigens present on all RBCs except those of the
Mcleod phenotype
Who described Mcleod phenotype
Allen and his coworkers
Rare and are common in males via inheritance of X- linked through a carrier mother
Mcleod phenotype
The McLeod phenotype RBC lacks ___ and ___, with decreased expression of other Kell antigens
Kx and Km
a neuroacanthocytosis syndrome
Mcleod syndrom
Acanthocytosis, Reticulocytosis, Bilirubinemia, Low haptoglobin, and Splenomegaly
Mcleod syndrome
From a multiply transfused hemophiliac who in 1950 was found out to produce antibodies against an antigen named “Fya”
Duffy
They reported that majority of the African americans tested were Fy (a- b-). The gene responsible for such result was “Fy” - the gene is rare in whites
Sanger and colleagues in 1955
The Duffy genes is located at what chromosome
Chromosome 1
There are three alleles at the Fy locus
Fya, Fyb, Fy