Lutheran and Kell Blood Group System

Question 1

Anti-Lu^a was first found in patients with

Answer 1

lupus

Question 2

What is the frequency of
Au^a
Au^b

Answer 2

Au^a: 80%

Au^b: 50%

Question 3

What are the four pairs of antithetical antigens in the Lutheran system (005)

Answer 3

Lu^a
Lu^b
Au^a
Au^b

Question 4

Lutheran genes are found on chromosome __ (long arm) and are linked to ______.

Answer 4

Chromosome 19
SE locus (secretor gene)

Question 5

The Lu glycoprotiein is a member of the ___ superfamily.

Answer 5

IgG superfamily

Question 6

Lutheran antigens are carried on two Type 1 integral membrane proteins called

Answer 6

Lu glycoprotein

Epithelial Cancer Antigen (B-CAM)

Question 7

The Lu glycoprotein is a receptor for the protein _____.

Answer 7

Laminin

Question 8

Laminin is a major component in

Answer 8

basement membranes

Question 9

Patients with sickle cell exhibit increased expression of this antigen

Answer 9

Lu

Question 10

What happens when sickle cell patients exhibit increased expression of Lu antigen?

Answer 10

Lu binds increasingly with laminen and may contribute to vaso-occlusion events in sickle cell patients

Question 11

What is the frequency of Lu^(b+)?

Answer 11

99.8%

Question 12

When do people fully develop Lu antigens?

Answer 12

15 years (poorly developed at birth)

Question 13

Do Lu antibodies show dosage effect?

Answer 13

Yes

Question 14

The Lutheran (005) null phenotype is expressed

Answer 14

Lu(a-b-)

Question 15

Lu(a-b-) is due to

Answer 15

In(Lu) dominant, recessive, and X-linked genetic causes

Question 16

People with InLu have decreased antigens in __ and __ systems

Answer 16

P1

I

Question 17

These antibodies may be naturally occuring, have room temperature reactions (IgM or IgG), may bind complement, have a mix field reaction, and cause mile HDFN.

Answer 17

Anti-Lu^a

Question 18

These antibodies are mostly IgG, cause mild HTR, and mild HDNF (since antigens are poorly developed at birth)

Answer 18

Anti-Lu^b

Question 19

anti-Lu3 can be made by individuals who are

Answer 19

Ly(a-b-)

Question 20

The Kell Blood Group systejm is designated by ISBT as

Answer 20

006

Question 21

Kell antigens were founded by

Answer 21

Coombs, Mourant, and Race

Question 22

How many antigens are in the Kell system?

Answer 22

24

Question 23

Kell antigens are found on the RBC as ____ pass type __ glycoprotein

Answer 23

single pass

type II

Question 24

The KEL gene is found on which chromosome

Answer 24

Chromosome 7

Question 25

This null phenotype can cause sever genetic defects

Answer 25

Ko (very rare)

Question 26

This phenotype expresses weak kell antigens and is not very well understood

Answer 26

Kell mod

Question 27

I the Kell Blood Group System K=Kell C= ?

Answer 27

C= Cellano

Question 28

At 10 weeks, this antigen can be found on fetal RBCs

Answer 28

K

Question 29

At 7 week, this antigen can be found on fetal RBCs

Answer 29

k

Question 30

Percentage of K antigens Caucasians? African Americans?

Answer 30

Caucasians: 9% | African Americans: 2%

Question 31

Do Kell antibodies show dosage effect?

Answer 31

Yes (react best with homozygous cells)

Question 32

Are Kell antigens destroyed by enzymes?

Answer 32

No

Question 33

This antigen is second to D antigen in immunogenicity

Answer 33

Kell

Question 34

What is the probability of developing anti-K after one transfusion?

Answer 34

10%

Question 35

99.8% of people have this antigen in the Kell Blood Group System (006)

Answer 35

k

Question 36

Js^b is a high frequency antigen of the Kell bloog group system found in

Answer 36

Caucasians | African Americans

Question 37

Js^a (Sutter) is found in 20% of this race

Answer 37

African American

Question 38

Kp^a (Penney) is found in 2% of this race

Answer 38

Caucasians (rare in African Americans)

Question 39

Kp^b (Rautenberg) is a high frequency antigen found in __ % of the population

Answer 39

99%

Question 40

2.6% of Finns and 0.46% of Japanese have this antigen

Answer 40

Ul^a

Question 41

Ku antigen is also refered to as

Answer 41

total Kell antigen

Question 42

If Ku antigen is lacking, a person is considered

Answer 42

Kell null

Question 43

Patiens who are K-, k-, Kp(a-b-) are considered

Answer 43

Ko or Kell null

Question 44

Ko or Kell null peopl express large amounts of

Answer 44

Kx antigen (cell morphology is normal)

Question 45

Kell antibodies are called

Answer 45

Anti-Ku | Anti-K5

Question 46

People with anti-Ku or anti- K5 must be giving

Answer 46

antigen negative blood

Question 47

Which 2 substances can be used to make artificial Ko cells for testing?

Answer 47

ZZAP (cystine-activated papain and dithiothreitol) AET (2- aminoethylisothiouronium)

Question 48

Who discovered the McLeod pehnotype?

Answer 48

Allen and colleagues

Question 49

In the McLead phenotype, RBCs of Hugh McLeoad had markedly decreased expression of _, _, and _.

Answer 49

k Js^b Kp^b

Question 50

McLeod phenotype is rare and only affects ___. Anti-___ can be formed.

Answer 50

Males | Anti-Kx

Question 51

In the McLeod phenotype, what is absent?

Answer 51

XK glycoprotein

Question 52

In the McLeod phenotyp, missing XK glycoproteins leads to weakend expression of ...

Answer 52

All Kell antigens

Question 53

Abnormal RBC morphology, irregular shaped and protrusions, some compensated hemolytic anemia, some muscular abnormalities, cardiomyopathy, and increads CK-MM are all symptoms of...

Answer 53

The McLeod phenotype

Question 54

This phenotype is a result of an X-linked granulomatous disease (CGD)

Answer 54

McLeod

Question 55

What is a key feature of a X-linked granulomatous disease (CGD)?

Answer 55

Granulocytes can ingest bacteria and fungi, but not kill them

Question 56

What are some examples of X-linked diseases?

Answer 56

``` McLeod phenotype Retinitis pigmentosa (RP) Duchenne-type muscular dystrophy (DMD) ```

Question 57

This results in weakens expression of ALL Kell antigen

Answer 57

Kp^a in cis position

Question 58

This results in weakend expression of all HIGH INCIDENCE Kell antigens

Answer 58

Kmod

Question 59

The K antigen is the most immunogenic, following

Answer 59

ABO andRh antigens

Question 60

Kell system antigens are reactive in the ____phase

Answer 60

AHG phase (sometimes direct agglutination)

Question 61

20% of these antigens bind complement

Answer 61

Kell

Question 62

What might enhance Kell antigen reactions?

Answer 62

PEG | LISS

Question 63

These antigens might produce server HDNF and HTF, but early (before the cells produce hemoglobin)

Answer 63

Kell antibodies

Question 64

This is a rare form of anti-K

Answer 64

IgM

Question 65

This antibody is so rare, it is hard to kind units

Answer 65

Anti-k (K+-)

Question 66

To find units for rare Kell antigens (Kp^a, Kp^b, Kp^c, Js^a, Js^b) you must consider

Answer 66

race