BB LEC (Semis- Other Blood Groups- Quiz 2) Flashcards
Found and soluble in plasma and saliva
Lewis Blood Group
“Plasma antigen”
Lewis Blood Group
“Not” a true blood group antigen because it is acquired from the body tissues/Secretions
Lewis Blood Group
Expression of the Lewis antigens are influenced by? (2pts)
Hh and Sese genes
This blood group is weak during pregnancy
Lewis Blood Group
When was Lewis blood group first reported?
1946
Lewis blood group is named after the?
one of the first individuals to make the antibody
In Lewis Blood group, the antibody, Anti-Le^a, is an agglutinated RBCs from about ______ of English people
25%
Are the antigens in Lewis Blood group antithetical?
NO
It was believed that both antigen were antithetical but proven otherwise
Le genes (Le/FUT3) are located at the short arm of?
chromosome 19p13.3
The Le gene of Lewis blood group is linked to? (2pts)
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. These genes are:
H, Se, Le genes
The two alleles at the secretor locus (Lewis blood group):
Se and se
The two alleles at the Lewis locus:
Le and le
Le gene codes for?
L-fucosyltransferase
The L-fucosyltransferase that is coded by the Le gene adds fucose to ____________________ to form Leª and fucose to _________________ to form Leᵇ
N-acetylglucosamine of Type-1 precursor; H structure
Lewis typing may be impossible until about?
6 years of age
This was formed by Se by adding fucose to terminal galactose on type 1 precursor
H structure
These antigens are also found on lymphocytes and platelets
Lewis antigens
This Lewis antigen is from the genetic interaction of LeLe and Sese
Lewis Le (a-b+)
Lewis antigens are resistant to treatment by enzymes…?`
Ficin and Papain, Dithiothreitol, and Glycine-acid EDTA
Le gene adds “L-fucose” to the ______________ and;
H gene adds “L-fucose” to ________of the precursor substance forming the Leᵇ antigen
N-acetylglucosamine; galactose
Le gene adds _________ to the N-acetylglucosamine of the type 1 precursor substance to form the Leª
“L-fucose”
This is secreted regardless of secretor’s status
Leª substance
The antibodies from this blood group are IgM and Naturally occurring
Lewis Blood Group
The antibodies from this blood group react at room temperature and at 37C
Lewis Blood Group
The year the antithetical “k” or “Cellano” was first described by Levine and group
1949
The antibodies from this blood group react at immediate spin, bind complement, trigger in-vitro hemolysis, and do not cause HDFN
Lewis Blood Group
The year Kpª antigen and the null phenotype (Ko) were first described
1957
The year Jsᵇ antigen was first described (antithetical to Jsª, and both are related to Kell system)
1963
The year the antibody Anti-K was first identified in the serum of Mrs. Kelleher. Discovery by Robin Coomb
1946
The year the low-incidence antigen K24 was found
1985
The year both Jsª and Jsᵇ were officially added to the Kell system
1965
The year Jsª were first described, named after the first producer, “John Sutter”
1958
The year Kpᵇ was first described
1958
The year the K11 antigen was first reported
1971
The year the Kpᶜ antigen joined the Kell system
1979
The Kel gene is found on _____________ and consist of _________ exons
chromosome 7q33/34; 19 exons
5 sets of alleles that produce the Kell system’s antithetical antigens exist within the Kell locus and these are:
- K and k
- Kpª and Kpᵇ
- Jsª and Jsᵇ
- K11 and K17
- K14 and K24
The different Kell antigens are due to ____________ that results in amino acid substitutions
single base mutations
The Kell Antigens are located on a _________ glycoprotein with ______ amino acids
type II; 719 amino acids
Kell glycoprotein of the Kell antigens is a member of what family?
Neprilysin family
The Kell antigen expression is dependent on the presence of?
Xk protein
The N-terminal domain of the kell glycoprotein is intracellular, and the large external C-terminal domain is highly folded by ___________ and has _______ amino acids with ______ cysteine
residues
disulfide linkages; 665; 15
The kell glycoprotein is linked with XK protein by a disulfide bond at ______ of Kell protein, to the _________of the XK glycoprotein
Cys72; Cys347
- Found only on RBCs
- Appears on fetal red cells earlier than Rh proteins
Kell Antigens
Can be detected on fetal RBCs as early as 10 weeks gestation and is fully developed at birth
K antigen “Kell”
The Kell glycoprotein has been characterized as a ____________ which is central to zinc binding and catalytic activity
Zinc endopeptidase
- With 3,500 to 18,000 sites per RBC
- Low prevalence antigen
K antigen “Kell”
k antigen “Cellano” can be detected as early as?
7 weeks
Very immunogenic (second only to D)
K antigen “Kell”
Other term for:
- Kpᵇ antigen
- Kpª antigen
- Kpᵇ antigen- “Rautenberg”
- Kpª antigen “Penny”
Gene encoding for this antigen is associated with suppression of other Kell antigens
Kpª antigen
Found in 2% of whites
Kpª antigen
Found in 20% of blacks
Jsª antigen
The K0 - Kell null Phenotype was identified in what year and by whom?
Bruce Chown, Marion Lewis, and Kiroko Kaita in 1957
Resistant to ficin and papain, and are sensitive to trypsin and chymotrypsin. This is destroyed by Glycine-acid EDTA
Kell Antigens
A patient’s red cells lack the entire Kell glycoprotein, therefore, no Kell antigen
K0 - Kell null Phenotype
This reduces the disulfide bonds of the protein
Sulfhydryl reagents (such as 2-mercaptoethanol, Dithiothreitol, 2-aminoethyl-isothiouronium bromide)
The most commonly encountered antibody next to ABO and Rh
Anti-K
Associated with severe HTRs and severe HDFN
Anti-K
- Usually IgG (Predominantly IgG1) and reacts at AHG phase (IAT)
- Stimulated by pregnancy or transfusion and persists for years
Anti-K
Patients with this antibody should receive antigen-negative
Anti-K
Are not commonly detected because individuals who lack these high-incidence antigens/antibodies (?) are scarce
Anti-Kpª, Anti-Kpᵇ, Anti-Kpᶜ, Anti-Jsª, Anti-Jsᵇ
Antigens that are only found in Asians
Kpᶜ
Found in erythroid tissues, brain, lymphoid organs, heart, skeletal muscle
Kx Blood Group “Xk”
Discovered in a young male medical student in 1961. He was initially a Kell null but showed weak expression to some Kell antigens
McLeod Phenotype
True or False: The XK gene that encodes for the Xk protein is dependent to KEL gene
False. Xk protein is independent to KEL gene
Kx antigen is present on all RBCs except those of?
Mcleod phenotype
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
People with McLeod phenotype are known to have the?
McLeod Syndrome (a
neuroacanthocytosis syndrome)
Individual with McLeod Syndrome present…? (2pts)
Muscle and Nerve disorders
- Muscle dystrophy - with increase CK-MM and Carbonic anhydrase III
- Cardiomegaly
- Chronic Granulomatous Disease
- Acanthocytosis, Reticulocytosis, Bilirubinemia, Low haptoglobin, and Splenomegaly
McLeod Syndrome
A multiply transfused hemophiliac who in 1950 was found out to produce antibodies against an antigen name Fyª
Mr. Duffy (Duffy Blood Group)
In what year did Sanger and colleagues report that majority of the African Americans tested were Fy (a-b-)
1955
Most commonly encountered Duffy antigens
Fyª and Fyᵇ
Can be detected at 6 weeks gestation and are fully developed at birth
Duffy antigens
In what year did Ikin et. al describe the Anti-Fyᵇ in the serum of Mrs. Hahn following the birth of her third child
1951
In 1975, it was observed that _______ RBCs are resistant to Plasmodium knowlesi and Plasmodium vivax
Fy(a-b-)
There are about ________to ________ Fyª and Fyᵇ sites on Fy(a+b-) and Fy(a-b+) RBCs respectively
13,000 to 14,000
Found in other body tissues such as the brain, Colon, Endothelium, Lungs, Spleen, Thyroid, Thymus, and Kidneys
Duffy antigens
These antigens are destroyed by proteolytic enzymes (Ficin, Papain, Bromelin, Chymotrypsin)
Duffy antigens
Duffy Antigens reside on a glycoprotein of?
336 amino acids
The glycoprotein of Duffy antigen is predicted to traverse the cell membrane ______ times and with _____ predicted disulfide bridges
seven; two
Duffy glycoprotein is a member of the superfamily of chemokine receptors known as?
“Duffy antigen receptor for chemokines”
The amino acid of:
- Fyª
- Fyᵇ
- Fyª - glycine
- Fyᵇ - aspartic acid
The amino acid at _________ on Duffy glycoprotein defines Fyª and Fyᵇ polymorphism
position 42
Binds variety of proinflammatory cytokines
“Duffy antigen receptor for chemokines”
The gene of Duffy antigens is located near the centromere on the long arm of?
chromosome 1q23.2
The Duffy gene was linked to a visible, inherited abnormality of?
chromosome 1
The three alleles at the Fy locus
Fya, Fyb, and Fy
These encodes Fyª and Fyᵇ
Fyª and Fyᵇ
A duffy antigen that is a major allele in blacks
Fy
Duffy Antibodies are usually _____ and reacts at _____
IgG; AHG
Common and usually in a mixture of antibodies
Anti-Fyª
- Enhanced in a low ionic strength medium
- Rarely binds complement
Duffy Antibodies
Discovered in 1951 from an antibody detected in the serum of Mrs. Kidd who had in infant with HDFN
Kidd Blood Group
The antithetical partner of Jkª was found on?
1953
In what year was the null phenotype Jk(a-b-) was described?
1959
This antibody is present on RBCs positive for Jkª or Jkᵇ
Jk3
These antibodies are a significant cause of Hemolytic transfusion reactions
Kidd antibodies
- Found in most populations
- Both are well developed at birth
Jkª and Jkᵇ
Jkª can be detected on fetal RBCs at _______ gestation while Jkᵇ can be detected at _______ gestation
11 weeks; 7 weeks
The Jk gene is located on?
chromosome 18q12.3
These antigens are not found on other blood cells
Jkª and Jkᵇ (Kidd antigens)
Phenotypes of the Kidd Blood group
- Jk(a+b-)
- Jk(a+b+)
- Jk(a-b+)
- Jk(a-b-)
Resist lysis in 2M Urea and is associated with a dominant gene In (Jk)
Jk(a-b-)
“Null”. Lacks the 3 antigens. Common in Filipinos, Chinese, Japanese, and Indonesians
Jk(a-b-)
Weak and demonstrates dosage (reacts strongly with RBCs with double dose antigen)
Anti-Jkª and Anti-Jkᵇ (Kidd antibodies)
Found in combination with other antibodies
Anti-Jkª and Anti-Jkᵇ (Kidd antibodies)
Antibody reactivity of Anti-Jkª and Anti-Jkᵇ can be enhanced by using?
LISS or PEG
Stimulated by pregnancy or transfusion
Anti-Jkª and Anti-Jkᵇ (Kidd antibodies)
Known to be notorious for its danger in causing severe/fatal delayed HTR and some HDFN
Anti-Jkª and Anti-Jkᵇ
- An IgG that reacts at AHG phase
- Associated with both immediate and delayed HTRs and mild HDFN
Anti-Jk3
The antigen for that agglutinin is “I” is for?
“individuality”
I Blood Group was discovered by? and in what year?
Discovered by Weiner and his coworkers in 1956
They discovered the anti-i
Marsh and Jenkins
“I” is a _________ carbohydrate while “i” is a ________ carbohydrate, both are formed
due to the activity of glycosyl transferase
branched; linear
The reactivity of Anti-Jk3 is enhanced by?
enzyme pretreatment of the RBCs
Infant RBCs are rich in what “I” antigen?
“i”
- Both are high-prevalence antigens
- Expressed in a reciprocal relationship
I and i Antigens
This antigen is undetectable in infant RBCs
“I” antigen
During the first ________ of life, the quantity of “i” decreases
18 months
Adult RBCs are rich in what “I” antigen?
“I”
”i” antigen is trace
These antigens are precursors for the synthesis of ABO and Lewis antigens
I and i antigens
This antigen has more and more adult i present in adult RBCs compared to cord blood RBCs
“Adult-i”
Adults that retain their “i” antigen
“Adult-i”
“i” antigen activity is defined by at least _____ repeating N-acetyllactosamine units in linear form
two
Encodes the N-acetylglucosaminyltransferase that adds N- Acetylglucosamine (G1cNAc) to form the branches
IGnt gene (GCNT2)
This antigen activity is associated with a branched form of “i”
“I” antigen
found on chromosome 6p24
IGnt gene (GCNT2)
Other sources of I and i antigens:
- Membranes of leukocytes and platelets
- Also found in serum, saliva, human milk, amniotic fluid, urine, and ovarian cyst fluid
Diseases that can alter the expression of I and i antigens and can cause increased “i” antigen expression.
(State at least 3)
- Acute Leukemia
- Hypoplastic anemia
- Megaloblastic anemia
- Sideroblastic anemia
- Thalassemia
- Sickle cell disease
- Paroxysmal nocturnal hemoglobinuria
- Chronic hemolytic anemia
- Not associated with in-vivo RBC destruction
- Weak, naturally occurring, saline-reactive IgM agglutinin
- Stronger antibodies agglutinate test cells at room temperature and binds complement
Benign antibody of Anti-I
Common autoantibody found in virtually all sera
Anti-I
True or False: Antigens (“I” and “i”) in secretions do not correlate with RBC expression and are thought to develop under separate genetic control
TRUE
How can you enhance anti-i reactivity?
By incubating in cold
Detected by polyspecific AHG reagent
Benign antibody of Anti-I
- With strong agglutination to adult RBCs (weak with cord blood or adult i RBCs)
- Production can be stimulated by microorganisms carrying I-like antigen
Anti-I
Tested at 4C and with enzyme treated RBCs
Anti-I
Anti-I production can be stimulated by microorganisms carrying I-like antigens except in patients with?
M. pneumoniae infection
Patients with this infection may experience acute abrupt hemolysis as the infection begins
Patients with M. pneumoniae infection
A strong IgM agglutinin with higher titers and a broad thermal range of activity (may reach 30-32C)
Pathogenic autoanti- I
In pathogenic autoanti-I, when the circulation cools in response to low ambient temperatures, the antibodies will attach in-vivo and cause _________ and a ______________
autoagglutination; Vascular occlusion, or hemolytic anemia
Reacts with adult and cord RBCs at room temperature and at 4C
Pathogenic autoanti- I
A rare antibody that gives strong reactions with cord RBCs and adult-i RBCs
Autoanti-i
Exist as IgG or IgM in the serum of most Adult-i individuals
Alloanti-I
Not common in healthy individuals
Autoanti-i
- With weaker reactions to adult RBCs
- Most are IgM and react with saline suspended cells and at 4C
- IgG types are rare but are associated with HDFN
Autoanti-i
Some patients with autoanti-i are associated with? (2pts)
Infectious mononucleosis and lymphoproliferative
disorder
The P blood Group or “P1PK” is comprised with?
P, P1, Pᵏ, and Luke
These antigens are not considered as a single blood group
P, P1, PK, Luke
These antigens are assigned to the Globoside blood group system (028)
P and PX2
These antigens are assigned to the P1PK blood group system (003)
P1 and Pᵏ
This antigen is assigned to the Globoside collection (209)
LKE
Introduced in 1927 by Landsteiner and Levine
P blood Group or “P1PK”
Discovered via injection of human RBCs into rabbits which produced new antibodies
P blood Group or “P1PK”
In the P blood Group or “P1PK” the antibody was initially called?
“Anti-P”
The finding of Anti-P divided the human RBCs into?
“P+” or “P-”
An Antibody to a high-prevalence antigen that Sanger later showed was related to the P blood group
anti-PP1Pᵏ
In what year did Levine and his colleagues describe Anti-Tjª which is now known as
anti-PP1Pᵏ?
1951
Sanger influenced the renaming and formation of the P blood group system
- P+ became _____
- P- became _____ and
- rare P null became ______
- P+ became “P₁”
- P- became “P₂” and
- rare P null became “p”
In 1959, __________ described the antigen Pᵏ
Matson and coworkers
The antigen is expressed on all RBCs except those of very rare p phenotypes. Not readily detected unless P is absent
Antigen Pᵏ
The 2 common phenotypes of P Blood Group
P1 and P2
Describes RBCs that react with Anti-P1 and Anti-P
P1
Describes RBCs that do not react with Anti-P1 but react with Anti-P
P2
The 3 rare phenotypes of P Blood Group
p, P₁ᵏ, and P₂ᵏ
This phenotype does not react with Anti-P1, Anti-P, or Anti-Pᵏ
“p”
Reacts with Anti-Pᵏ but does not react with Anti-P1 or Anti-P
P₂ᵏ phenotypes
These phenotypes are rare and are common in Japan, North Sweden, and in Ohio
“p” phenotypes (P null)
Reacts with Anti-P1 and Anti-Pᵏ but not with Anti-P
P₁ᵏ phenotype
The P Blood Group Antigens are synthesized by?
glycosyltransferases
adds sugar to a precursor substance
This P blood group antigen can be found on platelets, epithelial cells, and fibroblasts
“P”
These P Blood Group Antigens have been found in plasma as glycosphingolipids and
glycoproteins in hydatid cyst fluid
P and Pᵏ
The reactivity of these antibodies is greatly enhanced by testing with enzyme-treated RBC
P antibodies
Poorly expressed at birth (may even take 7 years for full expression)
P1 antigen
The Antigen strength of P1 antigen varies from?
inheritance to Race
Pᵏ synthesis is carried out by?
4- α- galactosyltransferase (Gb3, Pᵏ synthase)
Deteriorates rapidly on storage
P1 antigen
Blacks have stronger expression of _____ than whites
P1
Strong antibodies to P1 have also been found in patients with __________
Fascioliasis
The common precursor of P antigen
Lactosylceramide (Gb2, Ceramide
dihexose, or CDH)
The discovery of anti-P1 in two P1-individuals infected with _____________ led to the discovery of P1 and Pk substances in _____________
Echinococcus
granulosus worms; hydatid cyst fluid
Differences among P1+ individuals may be controlled __________ or via _______________
genetically; homozygous/heterozygous inheritance
This is a bovine liver fluke disease
Fascioliasis
This converts Pᵏ to P
β-N- acetylgalactosaminyltransferase (Gb4 synthase)
P synthesis formation is carried out by?
3-β-N- acetylgalactosaminyltransferase (Gb4 synthase)
This gene is located on chromosome 3q25 and encodes the 3-B-N- acetylgalactosaminyltransferase
Globoside gene (B3GALNT1)
Tippett and colleagues described an antibody in the serum of a ______________ in 1965
Hodgkin’s lymphoma patient
This gene is located on chromosome 22q13.2 and encodes the 4-a- galactosyltransferase
P1PK gene (A4GALT)
Luke antigens (LKE) divided the population into three phenotypes:
- 84% = Luke (+)
- 14% = Luke (w) (means weakly positive)
- 2% = Luke (-)
All p and Pᵏ phenotypes are?
Luke (-)
The group under P Blood group since the antibody reacted in all RBCs except 2% of P1 and P2 phenotypes
Luke Antigens (LKE)
Common, naturally occurring IgM antibody in the serum of P1(-) individuals and is poorly developed on fetal RBCs
Anti-P1
Anti-PP1Pᵏ was first discovered in the serum of Mrs. Jay, a “p” individual with?
adenocarcinoma of the stomach
Formerly “Anti-Tjª”
Anti-PP1Pᵏ
Reaction varies especially if old RBCs are used
Anti-P1
- Weak, cold-reactive saline agglutinin (reacts at 4C)
- Stronger types react at room temperature
- Rare types react at 37 C and bind complement
- These types are detected at the AHG phase (polyspecific reagent)
- May cause in-vivo RBC destruction, immediate or delayed HTRs
Anti-P1
Produced by “p” individuals early in life without RBC sensitization and reacts with all RBCs except those of the “p” phenotype
Anti-PP1Pᵏ
A component of anti-PP1Pᵏ in “p” individuals and is naturally occurring in the sera of Pᵏ individuals
Alloanti-P
A P Blood group antibody that is rare and IgMs that reacts at room temp
Anti-LKE
- Can be separated by adsorption
- Reacts over a wide thermal range and bind cocomplement
anti-P, anti-P1, and anti-Pᵏ
Produced early in life without RBC sensitization; Can cause
HTRs and HDFN
Anti-PP1Pᵏ
HLA detectable on RBCs
Bennet-Goodspeed Antigens
Enumerate the Bennet-Goodspeed Antigens
- Bgª antigen = HLA-B7
- Bgᵇ antigen = HLA-B17
- Bgᶜ antigen = HLA-A28
IMPORTANCE OF STUDYING OTHER BLOOD GROUP SYSTEM (FINALLLYYY!!!!)
- Avoid Hemolytic Transfusion Reactions and Hemolytic Disease of the Fetus and
the Newborns - Avoid confusion brought up by insignificant antibodies
- Avoid mistyping
- Properly find and identify potential donors to rare blood group patients
