Lutheran and Kell Blood Group System Flashcards

1
Q

Anti-Lu^a was first found in patients with

A

lupus

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2
Q

What is the frequency of
Au^a
Au^b

A

Au^a: 80%

Au^b: 50%

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3
Q

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

A

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

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4
Q

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

A
Chromosome 19
SE locus (secretor gene)
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5
Q

The Lu glycoprotiein is a member of the ___ superfamily.

A

IgG superfamily

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6
Q

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

A

Lu glycoprotein

Epithelial Cancer Antigen (B-CAM)

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7
Q

The Lu glycoprotein is a receptor for the protein _____.

A

Laminin

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8
Q

Laminin is a major component in

A

basement membranes

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9
Q

Patients with sickle cell exhibit increased expression of this antigen

A

Lu

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10
Q

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

A

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

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11
Q

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

A

99.8%

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12
Q

When do people fully develop Lu antigens?

A

15 years (poorly developed at birth)

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13
Q

Do Lu antibodies show dosage effect?

A

Yes

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14
Q

The Lutheran (005) null phenotype is expressed

A

Lu(a-b-)

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15
Q

Lu(a-b-) is due to

A

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

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16
Q

People with InLu have decreased antigens in __ and __ systems

A

P1

I

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17
Q

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.

A

Anti-Lu^a

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18
Q

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

A

Anti-Lu^b

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19
Q

anti-Lu3 can be made by individuals who are

A

Ly(a-b-)

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20
Q

The Kell Blood Group systejm is designated by ISBT as

A

006

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21
Q

Kell antigens were founded by

A

Coombs, Mourant, and Race

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22
Q

How many antigens are in the Kell system?

A

24

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23
Q

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

A

single pass

type II

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24
Q

The KEL gene is found on which chromosome

A

Chromosome 7

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25
Q

This null phenotype can cause sever genetic defects

A

Ko (very rare)

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26
Q

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

A

Kell mod

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27
Q

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

A

C= Cellano

28
Q

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

A

K

29
Q

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

A

k

30
Q

Percentage of K antigens
Caucasians?
African Americans?

A

Caucasians: 9%

African Americans: 2%

31
Q

Do Kell antibodies show dosage effect?

A

Yes (react best with homozygous cells)

32
Q

Are Kell antigens destroyed by enzymes?

A

No

33
Q

This antigen is second to D antigen in immunogenicity

A

Kell

34
Q

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

A

10%

35
Q

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

A

k

36
Q

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

A

Caucasians

African Americans

37
Q

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

A

African American

38
Q

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

A

Caucasians (rare in African Americans)

39
Q

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

A

99%

40
Q

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

A

Ul^a

41
Q

Ku antigen is also refered to as

A

total Kell antigen

42
Q

If Ku antigen is lacking, a person is considered

A

Kell null

43
Q

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

A

Ko or Kell null

44
Q

Ko or Kell null peopl express large amounts of

A

Kx antigen (cell morphology is normal)

45
Q

Kell antibodies are called

A

Anti-Ku

Anti-K5

46
Q

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

A

antigen negative blood

47
Q

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

A

ZZAP (cystine-activated papain and dithiothreitol)

AET (2- aminoethylisothiouronium)

48
Q

Who discovered the McLeod pehnotype?

A

Allen and colleagues

49
Q

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

A

k
Js^b
Kp^b

50
Q

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

A

Males

Anti-Kx

51
Q

In the McLeod phenotype, what is absent?

A

XK glycoprotein

52
Q

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

A

All Kell antigens

53
Q

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

A

The McLeod phenotype

54
Q

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

A

McLeod

55
Q

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

A

Granulocytes can ingest bacteria and fungi, but not kill them

56
Q

What are some examples of X-linked diseases?

A
McLeod phenotype
Retinitis pigmentosa (RP)
Duchenne-type muscular dystrophy (DMD)
57
Q

This results in weakens expression of ALL Kell antigen

A

Kp^a in cis position

58
Q

This results in weakend expression of all HIGH INCIDENCE Kell antigens

A

Kmod

59
Q

The K antigen is the most immunogenic, following

A

ABO andRh antigens

60
Q

Kell system antigens are reactive in the ____phase

A

AHG phase (sometimes direct agglutination)

61
Q

20% of these antigens bind complement

A

Kell

62
Q

What might enhance Kell antigen reactions?

A

PEG

LISS

63
Q

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

A

Kell antibodies

64
Q

This is a rare form of anti-K

A

IgM

65
Q

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

A

Anti-k (K+-)

66
Q

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

A

race