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Carbohydrate Antigens Flashcards

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1
Q

Enzyme that converts i to I

A

B-1,6-acetylglucosaminyltransferase

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2
Q

Gene

I

A

IGnT or GCNT2

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3
Q

Number of antigen sites

I

A

32,000-500,000

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4
Q

Number of antigen sites

i

A

20,000-70,000

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5
Q

Location in RBC membrane:

I/i

A

Glycoproteins, glycolipids, Band 3

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6
Q

Present in which body fluids:

I/i

A 
Saliva
Milk
Amniotic fluid
Urine
Ovarian cyst
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7
Q

Present on which other blood cells:

I/i

A

Lymphocytes
Monocytes
Platelets
Granulocytes

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8
Q

Expression on adult cells:

I/i

A

I: strong

i: weak

Complete conversion around age 2

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9
Q

Expression on cord cells:

I/i

A

I: weak

i: strong

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10
Q

i adult phenotype without cataracts

A

Autosomal recessive

Mutation in exon 3 of GCNT2

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11
Q

Auto anti-I

Ig class

A

IgM

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12
Q

Auto anti-I

Temp of reactivity

A

RT to 4C- benign

30-37: pathogenic

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13
Q

Auto anti-I

Bind complement

A

Rarely

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14
Q

Auto anti-I

Hemolysis in vitro

A

No

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15
Q

Auto anti-I

Reactivity with enzyme treated RBCs

A

Increased

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16
Q

Auto anti-I

HTR/HDFN

A

No

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17
Q

Alloanti-I

Ig class

A

IgM

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18
Q

Alloanti-I

Bind complement

A

Rarely

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19
Q

Alloanti-I

Hemolysis in vitro

A

No

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20
Q

Alloanti-I

Temp of reactivity

A

RT - 4 C

Rarely 37

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21
Q

Alloanti-I

Reactivity with enzyme treated RBCs

A

Increased

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22
Q

Alloanti-I

HDFN

A

No

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23
Q

Alloanti-I

HTR

A

None to rare

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24
Q

Auto anti-i

Ig class

A

IgM

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25
Auto anti-i Temp of reactivity
RT-4C: benign | 30-37: pathologic
26
Auto anti-i Bind complement
Rarely
27
Auto anti-i Hemolysis in vitro
No
28
Auto anti-i Reactivity with enzyme treated RBCs
Increased
29
Auto anti-i HTR/HDFN
No
30
Chain type of ABH In secretions Also adsorbed onto RBCs
Type 1 chain
31
Chain type of most ABH on RBCs
Type 2 chain
32
H gene (FUT1) encodes
a-2-L-fucosyltransferase
33
H antigen expression
O>A2>B>A2B>A1>A1B>Bombay
34
ABO antibodies Detection age
3-6 months
35
ABO antibodies Adult levels reached
5-10 years
36
ABO antibodies Titers
Vary from 4- >2048 Anti-A titers higher than anti-B Titers higher in group O individuals than A or B
37
ABO antibodies Ig class
Mostly IgM Group Os have a mix of IgG and IgM
38
ABO antibodies Temp of reactivity
4C | IgG will also react at 37
39
ABO antibodies In vitro hemolysis
Yes (intravascular)
40
ABO antibodies HTR
Yes
41
ABO antibodies HDFN
Yes, usually mild
42
Lectin Bandeiraea simplicifolia
Anti-B
43
Lectin Dolichos biflorus
Anti-A1
44
Lectin Ulex europaeus
Anti-H
45
Lectin Phaseolus limensis
Anti-A
46
Lectin Helix pomatia
Anti-A Snails
47
Lectin Anguilla japonica
Anti-H Eel
48
Terminal epitope A antigen
N-acetylgalactosamine
49
Terminal epitope B antigen
Galactose
50
Terminal epitope H antigen
Fucose
51
ABO antigen age of detection
5-6 week gestation Adult levels 2-4 years (precursor chains branch with age allowing for more antigen expression)
52
ABO gene location
Chromosome 9q34
53
A1
80% of A donors Approx 5x the A than A2
54
A2
Most common subtype @ 20% Inefficient conversion of H Less A antigen more H
55
Acquired B
Deacetylation of the A antigen yields a B like galactosamine Typically shows weak agglutination with anti-B reagents w/ negative auto control
56
Acquired B disease associations
Infection by gastrointestinal bacteria
57
Acquired B resolution
Type patient cells with different monoclonal reagent or acidified (pH 6) anti-B
58
Anti-A1 frequency in A2 individuals
1-8% A2 | 22-35% A2B
59
Anti-A1 Ig class
Usually IgM
60
Anti-A1 Temp of reactivity
Usually RT or below- clinically insignificant Rarely 37- clinically significant
61
Lewis antigen present on which cells/tissues
Platelets Endothelial cells Kidney tissue Genitourinary and gastrointestinal epithelium
62
Lewis antigen on RBCs
NOT synthesized by RBCs Passively adsorbed onto the cell membrane May be eluted with increase plasma volume or increased circulating lipoprotein Levels decrease on stored RBCs
63
Lewis and secretor gene
Leb +
64
Lewis gene but no secretor gene
Lea+
65
Le(a+b+)
Transiently seen in infants while secretor activity increases Seen in Asian populations where weak secretor gene is common (18% Japanese)
66
Lewis expression in newborns
Le(a-b-) Valid phenotype not present til age 5-6
67
Lewis phenotype prevalence: European
Le(a+b-): 22% Le(a-b+): 72% Le(a-b-): 6% Le(a+b+): rare
68
Lewis phenotype prevalence: African
Le(a+b-): 23% Le(a-b+): 55% Le(a-b-): 22% Le(a+b+): rare
69
Lewis antibodies Ig class
Generally IgM
70
Lewis antibodies In Le(a-b+) individuals
Do not make anti-Lea as trace amount of Lea is synthesized
71
Anti-Leb
May show abo specificity Most common anti-Leb H
72
Lewis antibodies HDN
Unlikely Antigens readily shed from cells after transfusion Antigen negative RBCs generally not indicated for most patients
73
Lewis antibodies HDFN
No Predominantly IgM and antigen poorly expressed on neonate RBCs
74
GCNT2 (I) chromosome location
6p24
75
Difference between i adult w/cataracts and w/o cataracts
W/o cataracts mutation is in Exon 1c which is specific for I synthesis in RBCs (so none on red cells but present on other tissue) W/ cataracts is gene deletion or mutation in exon 2 or 3 meaning I synthesis is lost in all tissues
76
Anti-i disease association
Infectious mononucleosis
77
P1 and P2 phenotypes both express
Pk and P
78
Rare recessive phenotypes in P1PK/GLOB
P1k, P2k, p Associated with natural production of antibodies
79
P, Pk, P1 synthesis
Stepwise addition of sugars to lactosylceramide Pk synthesized first- precursor of all globo-series glycosphingolipids
80
Pk requires what gene
A4GALT
81
Pk is a substrate for what enzyme
B1-3-N-acetylgalactosaminyltransferase (B3GALNT1) forms P antigen
82
P1
A4GALT adds galactose to paragloboside
83
p phenotype
Mutation in A4GALT Loss of all globo family and P1
84
ABO discrepancies Missing/Weak antigen
subgroup of A/B | suppressed antigen
85
ABO discrepancies extra/unexpected antigen
acquired B B(A) antibody coating cells
86
ABO discrepancies missing/weak antibody
age Hypo or agammaglobuliemia Disease (Lymphomas, Waldenstrom's marcroglobuliemia, CLL, Immusuppressive, and Immudeficiency)
87
ABO discrepancy extra/unexpected antibody
``` rouleaux anti-A1 autoantibody cold alloantibody passively acquired antibody ```
88
A3
weakly positive with anti-A | mf
89
Aend
MF agglutination but very weak only 10% of cells agglutination No A substance in secretions Anti-A1 usually present
90
Ax
not agglutinated by Anti-A but sometimes weak with Anti-A,B human sera most monoclonal reagents detect similar to Am except A substance not in secretions Ax has substance that is only detected using Ax cells Anti-A can be adsorbed and eluted off
91
Am
negative with anti-A adsorbs/elutes anti-A or -A,B secretes A & H
92
Ay
negative with anti-A adsorbs/elutes anti-A or -A,B secretes weak A and H
93
Ael
negative with anti-A adsorbs/elutes anti-A or -A,B secretes only H
94
B(A)
super B gene produces a--3-N-acetylgalactosaminyltransferase extra "A like" antigen
95
Lewis gene/chromosome
FUT3 chromosome 19
96
P phenotypes frequency Caucasion
``` P1: 79% P2: 21% p: rare P1k: very rare P2k: very rare ```
97
P phenotypes frequency African American
``` P1: 94% P2: 6% p: rare P1k: very rare P2k: very rare ```
98
p (null phenotype)
lack P1, P, and Pk
99
High prevalence in P1PK/GLOB
Pk, P, PX2, LKE
100
P Asian frequencies:
``` P1: 20% P2: 80% p: rare P1k: rare P2k: rare ```
101
B3GALNT1 mutation
Pk phenotype Loss of P, LKE, and PX2
102
Anti-P1
Naturally occurring IgM present in 25-33% of P2 individuals
103
Anti-P1 HDFN/HTR
Rarely crossed placenta- no HDFN Rare reactivity at 37 or invitro hemolysis
104
P1 levels during storage
Decrease
105
P1 expression
Varies in strength among individuals Anti-P1 may not react with all P1 + cells
106
Anti-P1 with enzyme treated cells
Reactivity enhanced
107
Anti-P1 inhibited by:
Hydatid cyst fluid | P1 substance from pigeon eggs
108
Anti-PP1Pk (anti-Tja)
Separable mixture of the antibodies found in p people
109
Alloanti-P (in P1k, P2k, and p) characteristics
Naturally occurring Predominantly IgM or a mix of IgM and IgG Associated with hemolytic transfusion reaction and occasional HDFN ASSOCIATED WITH RECURRENT SPONTANEOUS ABORTIONS (placenta rich in P and Pk antigen)
110
Auto anti-P association
Paroxysmal cold hemoglobinuria Donath landsteiner test
111
FORS1 characteristics
Addition of N-acetylgalactosamine to the P antigen Resembles the A antigen and may be picked up by certain anti-A reagents (originally thought to be an A subgroup) Gene: GBGT1 Most people have naturally occurring anti-FORS1
112
How is Pk antigen formed?
A4GALT enzyme adds galactose to Lactosylceramide
113
How is P antigen formed?
P antigen is formed from Pk | B3GALNT-1 adds GalNAc to Pk > P antigen
114
How is NOR antigen formed?
Formed from P antigen | A4GALT enzyme adds galactose to P antigen
115
How is LKE antigen formed?
Formed from P antigen | A4GALT enzyme adds galactose and sialic acid is added to form LKE
116
Characteristics of P1 antigen
Weaker on cord cells until 10 years of age Antigen varies in strength and is weaker on older cells Enzymes enhance reactivity Not destroyed by Papain, Ficin, EGA, DTT, Chloroquine
117
What disease is Pk antigen associated with?
Receptor for shiga toxin Receptor for Streptoccocus suis
118
What disease is P antigen associated with
Receptor for parvovirus
119
Disease associated with anti-I
Mycoplasma pnuemoniae
120
Characteristics of pathological auto anti-I in CHD
Reacts at 32C and activates complement at 30-37C Titer is > 500 at 4C and >128 at room temp Will hemolyze enzyme treated cells at low pH Antibody is typically monoclonal or idiopathic
121
Characteristics of benign auto-I?
Reacts at <25C and activates Complement at <25C Titer is < 64 at 4C and <16 at room temp Antibody is polyclonal
122
Diseases and Ii concentration
Increased I and i is found in chronic hemolytic disorders and is a sign of stresses erythropoiesis - Thalassemia major - Hypoplastic anemia - CLL - Acute leukemia - Bombay phenotype
123
What chromosome is Le gene located on?
Chromosome 19
124
Leb is a receptor for...
H. pylori - bacteria responsible for causing gastritis Norovirus
125
What is Le(a-b-) associated with?
Increased susceptibility to E. Coli, candida, Cardiovascular disease, and possibly graft failure in renal transplant patients
126
What chromosome is H gene located on
Chromosome 19
127
What disease is also produces Oh phenotype
LAD - Leukocyte adhesion deficiency also produces Oh cells due to deficiency in GDP-fucose transporter
128
What is CIS AB?
non-mendelian inheritance 1 gene produces mutant enzyme that makes both A and B transferase Cis AB/O, Cis, AB/A, Cis, CisAB/B
129
Describe neutralization of Anti-A or Anti-B
Occurs with plasma suspended cells in patients with carcinoma of stomach or pancreas. Patients have an increased A or B substance that binds to Anti-A or -B reagent making antibody unable to bind to patient RBC ag
130
ABO frequency European
O: 45% A: 40% B: 11% AB: 4%
131
ABO frequency African American
O: 49% A: 27% B: 20% AB: 4%
132
H disease associations
Helicobacter pylori Norovirus
133
Anti-Le^ab
Inseparable anti-lea and -Leb

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