Kell Flashcards

1
Q

Kell system antigens:

A

36 antigens

7 pairs/ 1 triplet

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2
Q

Kell antigen location

A

CD238

Kell spans the membrane once with a large c-terminal domain outside the membrane

Linked by disulfide bond to Xk protein

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3
Q

Absence of Xk

A

Reduced expression of Kell glycoprotein and weakened Kell antigen

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4
Q

KEL gene location

A

Chromosome 7q33

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5
Q

XK gene location

A

chromosome Xp21.1

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6
Q

Kell phenotype prevalence

Whites

A

K-k+: 91%
K+k+: 8.8%
K+k-: 0.2%

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7
Q

Kell phenotype prevalence

Blacks

A

K-k+: 98%
K+k+: 2%
K+k-: rare

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8
Q

Kpa prevalence

A

2% in whites

Not present in black or Japanese

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9
Q

Jsa prevalence

A

20% in African American

Rare in white peoples

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10
Q

Kell antibodies

A

Primarily IgG

Don’t bind complement

Clinically significant

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11
Q

Anti-Kell HDFN

A

Associated with lower degree of hemolysis and fetal anemia predominantly from suppression of erythropoeisis

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12
Q

Ko phenotype

A

K null

Results from homozygosity for various KEL mutations

Make anti-Ku (KEL5) and anti-Km

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13
Q

Kmod

A

Show weak expression of Kell antigens (often only detectable by adsorption/elution)

Strong Kx expression

Make an antibody similar to Anti-Ku that reacts with everything except other Kmod

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14
Q

Kell weakened antigen expression

A

Kmod

absence of Gerbrich Ge2 and 3, Kpa in cis

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15
Q

McLeod syndrome

A

X-linked

Associated with acanthocytosis

Late onset muscular and neurological defects

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16
Q

McLeod with CGD will make which antibodies

A

Anti-Km

Anti-Kx

17
Q

McLeod w/o CGD make which antibodies

A

Anti-Km only

18
Q

Hematologic abnormalities with McLeod

A
Decreased RBC survival
Acanthacytosis
Reticulocytosis
Reduced serum haptoglobin 
Increased bilirubin 

Compensated anemia