[13] CHAPTER VI LESSON 1 Flashcards
DISCOVERY/CHARACTERISTICS OF OTHER MAJOR BLOOD GROUP SYSTEM
a. Kell Blood Group
b. Duffy Blood Group
c. Kidd Blood Group
d. Lewis Blood Group
e. MNSs Blood Group
f. Lutheran Blood Group
g. P Blood Group
h. I Blood Group
DISCOVERY/CHARACTERISTICS OF OTHER MAJOR BLOOD GROUP SYSTEM
a. Diego Blood Group
b. Cartwright Blood Group
c. Chido Blood Group
d. Xg Blood Group
e. Scianna Blood Group
f. Gerbich Blood Group
g. Milton Blood Group
h. Knops Blood Group
i. Bg Blood Group
j. Indian Blood Group
k. Others
ABO
ABO
001
MNS
MNS
002
P1PK
P1PK
003
Rh
RH
004
Lutheran
LU
005
Kell
KEL
006
Lewis
LE
007
Duffy
FY
008
Kidd
JK
009
Diego
DI
010
Yt
YT
011
Xg
XG
012
Scianna
SC
013
Dombrock
DO
014
Colton
CO
015
Landsteiner-Wiener
LW
016
Chido/Rodgers
CH/RG
017
H
H
018
Kx
XK
019
Gerbich
GE
020
Cromer
CROM
021
Knops
KN
022
Indian
IN
023
Ok
OK
024
Raph
RAPH
025
John Milton Hagen
JMH
026
I
I
027
Globoside
GLOB
028
Gill
GIL
029
Rh-associated glycoprotein
RHAG
030
Note: many of these functional relationships have been predicted based on [?] and remain under investigation.
molecular cloning studies
Glycosyltransferases
ABO, P1PK, Lewis, and H blood group systems
Structural relationship to Red Cell
MNS, Diego, and Gerbich blood group systems
Transport Proteins
Rh, Kidd, Diego, Colton, and Kx blood group systems
Complement Pathway Molecules
Chido/Rodgers, Cromer, and Knops blood group systems
Adhesion Molecules
Lutheran, Xg, Lansteiner-Wiener, and Indian blood group systems
Microbial Receptors
MNS, Duffy, P, Lewis, and Cromer blood group systems
Biologic Receptors
Duffy, Knops, and Indian blood group system
It was named after one of the first individuals to make the antibody, reported by Mourant in 1946.
Lewis Blood Group System
The Lewis (Le, FUT3) gene is located on
chromosome 19 (at 19p13.3).
The Secretor (Se, FUT2) gene is located on
chromosome 19 (at 19q13.3)
There are two alleles at the Lewis locus, [?], and there are two alleles at the secretor locus, [?]
Le and the amorph le
Se and the amorph se
Le gene must be present for a precursor substance to be converted to [?], but the Se gene must also be present for conversion to [?].
Lea
Leb
Are not expressed on cord RBCs and are often diminished on the mother’s RBCs during pregnancy.
Lewis Antigens: Lea and Leb
They are found on lymphocytes and platelets and on other tissues such as the pancreas, stomach, intestine, skeletal muscle, renal cortex, and adrenal glands.
Lewis Antigens: Lea and Leb
are resistant to treatment with the enzymes ficin and papain, DTT and glycine acid EDTA.
Lewis Antigens: Lea and Leb
are not intrinsic to RBCs but are on Type 1 glycosphingolipids that are passively adsorbed onto the RBC membrane from the plasma.
Lewis Antigens: Lea and Leb
Are IgM and have no clinical significance.
Lewis Antibodies: Anti-Lea and Anti-Leb
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
It can bind complement.
Lewis Antibodies: Anti-Lea and Anti-Leb
The most commonly encountered of the Lewis
Lewis Antibodies: Anti-Lea and Anti-Leb
is the receptor for Helicobacter pylori, a gram-negative bacterium associated with gastritis, PUD, gastric carcinoma, and the Norwalk virus.
The Leb antigen
Whites: 22
Blacks: 23
Le (a+b-)
Whites: 72
Blacks: 55
Le (a-b+)
Whites: 6
Blacks: 22
Le (a-b-)
Whites: Rare
Blacks: Rare
Le (a+b+)
Le (a-b+) red cell phenotype arises from the inheritance of
Le, Se, and H gene.
Lewis glycolipids are not detectable in plasma until about
10 days after birth.
Cord blood and RBCs from newborn infant’s phenotype as
Le (a-b-).
In children who inherit the both the Le and Se gene:
Le(a-b-) at birth
Le (a+b-) after 10 days
Le (a+b+) and finally Le (a-b+), the TRUE Lewis phenotype, after about 6 years.
In contrast, children who inherit Le and sese genes phenotype as:
Le (a-b-) at birth
Le (a+b-) after 10 days
Le (a+b-) phenotype persist throughout life.
Individuals with lele genes phenotype as [?] at birth and for the rest of their lives.
Le (a-b-)
Lewis antigens found in the secretions are
glycoproteins
Lewis antigens found in the plasma are
glycolipids
adsorb only glycolipids, not glycoproteins, onto membrane
Red cells
A person can be a nonsecretor (sese) and still secrete [?] into the body fluid.
Lea
Le sese H
Lea
Le (a+b-)
Le Se H
Lea Leb H
Le (a-b+)
lele sese H
None
Le (a-b-)
lele Se H
H
Le (a-b-)
Le sese hh
Lea
Le (a+b-)
Le Se hh
Lea
Le (a+b-)
lele sese hh
None
Le (a-b-)
lele Se hh
None
Le (a-b-)
are not alleles
Lea and Leb
Individuals who have a phenotype of [?] are not secretors with the exception of the Bombay phenotype.
Le (a+b-)
A Bombay phenotype (hh) cannot express the
Leb antigen
Adult red cells with a phenotype of [?] are very rare.
Le (a+b+)
Traditionally, the P blood group comprised the
P, P1 and Pk abtigens and later, Luke (LKE).
are assigned to the P1Pk blood group system (003, P1PK)
P1 and Pk
is assigned to the globoside blood group system (028, symbol GLOB)
P
are assigned to the Globoside Collection (029, GLOB)
LKE and PX2
P1PK gene is located at
chromosome 22q11.2
P gene is located at
chromosome 3q26.1
: Biosynthetic Pathways of the P blood group antigens
Biochemistry
There are two distinct pathways for the synthesis of the P blood group antigens.
Biosynthetic Pathways of the P blood group antigens
The common precursor is [?] (or Gb2, also known as ceramide dihexose or CDH).
lactosylceramide
The pathway on the figure’s left results in the formation of
paragloboside and P1
is also the type 2 precursor for ABH.
Paragloboside
The pathway shown on the figure’s right side leads to the production of the globoside series:
Pk, P, and Luke (LKE).
The P blood group was introduced in
1927 by Landsteiner and Levine in 1927
Matson and coworkers:
Pk
may be found on RBCs, lymphocytes, granulocytes, and monocytes
P1, P, or Pk
can be found on platelets, epithelial cells, and fibroblasts.
P
have also been found in plasma as glycosphingolipids and as glycoproteins in hydatid cyst fluid
P and Pk
The antigens have not been identified in secretions.
P Antigen
Poorly expressed at birth and may take up to 7 years to be fully expressed.
The P1 Antigen
It deteriorates rapidly on storage.
The P1 Antigen
Blacks have stronger expression of P1 than whites
The P1 Antigen
P Antibodies 2 Categories:
Clinically insignificant
Potently hemolytic
: Parasitic Infections
*Anti-P1
: Early abortions
*Anti-PP1Pk or anti-P
: PCH
*Autoanti-P
tors for P-fimbriated uropathogenic E.coli (causes UTI).
*The P system v: recep
: receptor for shiga toxins, which cause shigella dysentery and E.coli-associated HUS.
*The Pk antigen
is the receptor of human parvovirus B19 Pk provides some protection against HIV infection of peripheral blood mononuclear cells.
*P
Common, naturally occurring IgM antibody in the sera of P- individuals.
Anti-P1
Anti-P1 in 2 P1 individuals infected with Echinococcus granulosus tapeworms led to the identification of P1 and Pk substance in hydatid cyst fluid.
Anti-P1
Typically weak, cold reactive saline agglutinin optimally reactive at 4oC and not seen in routine testing.
Anti-P1
Associated with parasitic infections.
Anti-P1
Strong antibodies to P1 have also been found in patients with fascioliasis and in bird handlers.
Anti-P1
Originally called anti-Tja.
Anti-PP1Pk
It was first described in the serum of Mrs. Jay, a p individual with adenocarcinoma of the stomach.
Anti-PP1Pk
It has the potential to cause severe HTRs and HDFN
Anti-PP1Pk
It is also associated with an increased incidence of spontaneous abortions in early pregnancy.
Anti-PP1Pk
Rarely seen but it is very significant in transfusion.
Alloanti-P
IgG class anti-P may occur and has been associated with habitual early abortion.
Alloanti-P
Associated with the cold reactive IgG autoantibody in patients with PCH.
Autoanti-P Associated with Paroxysmal Cold Hemoglobinuria (PCH)
The IgG autoantibody in PCH is described as a biphasic hemolysin.
Autoanti-P Associated with Paroxysmal Cold Hemoglobinuria (PCH)
Antibody binds to RBCs in the cold
Autoanti-P Associated with Paroxysmal Cold Hemoglobinuria (PCH)
Via complement activation, the coated RBCs lyse as they are warmed to 37 degrees Celsius.
Autoanti-P Associated with Paroxysmal Cold Hemoglobinuria (PCH)
It typically does not react in routine test systems but is demonstrable only by the DonathLandsteiner Test.
Autoanti-P Associated with Paroxysmal Cold Hemoglobinuria (PCH)
Described by Tippett and colleagues in 1965 in the serum of a patient with Hodgkin’s Lymphoma.
Luke (LKE) Antigen
Luke (LKE) Antigen Three phenotypes:
80% tested Luke+
14% Lule (w)
2% Luke-
*All individuals with the p and Pk phenotype are
Luke-
Rare
p Phenotype
Slightly more common in Japan, North Sweden, and in an amish group in Ohio.
p Phenotype
Antigens Present: P1, P, Pk
Possible Antibodies: None
P1
Antigens Present: P,Pk
Possible Antibodies: Anti-P1
P2
Antigens Present: None
Possible Antibodies: Anti-PP1Pk
P
Antigens Present: P1,Pk
Possible Antibodies: Anti-P
P1k
Antigens Present: Pk
Possible Antibodies: Anti-P, anti-P1
P2k
Whites: 79%
Blacks: 94%
P1
Whites: 21%
Blacks: 6%
P2
Whites: Rare
Blacks: Rare
P
Whites: Very rare
Blacks: Very rare
P1k
P2k
: I for Individuality
Wiener and coworkers
: reported finding anti-i
Marsh and Jenkins
I and i antigens are found on the
membranes of leukocytes and platelets in addition to RBCs
I and i have also been found in the [?] of adults and newborns and in [?]
plasma and serum
saliva, human milk, amniotic fluid, urine, and ovarian cyst fluid
is associated with much greater i activity on RBCs than control cord RBCs.
Chronic dyserythropoietic anemia type II or HEMPAS
In Asians, the adult i phenotype has been associated with
congenital cataracts
Both [?] are high-prevalence antigens
I and I Antigens
Infant RBCs are rich in
i
is almost undetectable
I
During the [?], the quantity of i slowly decreases as I increases until adult proportions are reached.
first 18 months of life
Adult RBCs are rich in
I
Anti-I: Strong
Anti-i: Weak
Anti-IT: Weak
Adult I
Anti-I: Weak
Anti-i: Strong
Anti-IT: Strong
Cord
Anti-I: Weak
Anti-i: Strong
Anti-IT: Weakest
Adult i
It is a common autoantibody that can be found in virtually all sera.
Anti-I
Testing at 4oC and/or against enzyme treated RBCs may be required to detect the reactivity.
Anti-I
It is not associated with HDFN because the antibody is IgM, and the I antigen is poorly expressed on infant RBCs.
Anti-I
Found in the serum of many normal healthy individuals and is benign- it is not associated with in vivo RBC destruction.
Autoanti-I
Weak, naturally occurring, saline reactive IgM agglutinin.
Autoanti-I
The production of autoanti-I may be stimulated by microorganisms carrying I-like antigen on their surface.
Autoanti-I
Associated with Mycoplasma pneumoniae infections
Autoanti-I
Associated with Cold agglutinin syndrome
Pathogenic autoanti-I
When peripheral circulation cools in response to low ambient temperatures, these antibodies attach in vivo and cause autoagglutination and peripheral vascular occlusion (acrocyanosis) or hemolytic anemia.
Pathogenic autoanti-I
Typically reacts with adult and cord RBCs equally well at room temperature and at 4oC.
Pathogenic anti-I
Exists as an IgM and IgG antibody in the serum of most individuals with the adult I phenotype.
Alloanti-I
: never been described
Allanti-i
Potent examples are associated with Infectious mononucleosis (Epstein Barr virus infections) and some lymphoproliferative disorders.
Autoanti-i
have also been described and have been associated with HDFN.
IgG Anti-i
The agglutinin recognizes a transition state of I into I and designated the specificity IT (T for “Transition”).
IT Antigen and Antibody
Examples of IgM and IgG anti-I reacting preferentially at 37oC have also been found in patients with WAIHA, with a special association with Hodgkin’s disease.
IT Antigen and Antibody
