Kell,Lutheren,HLA Flashcards

1
Q

What antigens make up the Kell group? (6)

A
  • K
  • k
  • Kp(a)
  • Kp(b)
  • Js(a)
  • Js(b)
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2
Q

Where are Kell antigens expressed?

-What are alloantibodies capable of?

A

Mature RBCs and Erythroid precursors

-Suppressing erythropoiesis

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3
Q

The Kell antigens are expressed in covalent association with what antigen?

A

Kx antigen

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4
Q

What is the relative prevalence of K(KEL1), Kp(a), and Js(a)?

A
  • K(KEL1) - 9%
  • Kp(a) - 2%
  • Js(a) - 0.1%
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5
Q

Which Kell antigens are found in high frequency (99% of donors)

A
  • k
  • Kp(b)
  • Js(b)
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6
Q

What is k antigen also known as?

A

Cellano or KEL2

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7
Q

The Kell null phenotype results from what?

-What do RBCs express?

A

Homozygous inheritance of the amorph K0

-RBCs have no Kell antigens but have an abundance of Kx

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8
Q

McLeod phenotype:

  • Gene mutation
  • Inheritance
  • RBC finding
A

McLeod phenotype:

  • Kx encoding (XK) gene
  • X-linked Recessive
  • RBC show Acanthocytosis
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9
Q

The McLeod phenotype is frequently associated with what other diseases? (3)

A
  • Chronic Granulomatous disease
  • Muscular Dystrophy (late onset type; “neuroacanthocytosis”)
  • Retinitis Pigmentosa
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10
Q

Anti-K antibodies:

  • temp
  • class
  • acquisition
A

Anti-K antibodies:

  • Warm
  • IgG
  • Acquired through exposure
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11
Q

Kell antigens are ________ by enzymes.

A

Unaffected

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12
Q

Kell antigen expression is diminished by agents that dissolve what bonds?
-Very sensitive to? (3)

A

Sulfhydryl bonds

  • 2-mercaptoethanol
  • ZZAP
  • Dithiothrietol (DTT)
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13
Q

Kell related HDFN is characterized by what?

A

Suppression of erythropoiesis

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14
Q

Lutheran Antigens - Incidence.

-What are the phenotypes?

A

Lu(b) - High (99%)
Lu(a) - 7%

Lu(a-b+) - 93%
Lu(a+b+) - 7%

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15
Q

Lutheran antigen expression is increased on the surface of what type of RBCs?

A

Sickle Cells

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16
Q

Lutheran antigeniticity is ______ by enzymes.

A

Destroyed

17
Q

What else destroys Lutheran antigenicity?

A
  • 2-ME

- DTT

18
Q

Lutheran antibodies:

  • Specificity to
  • Temp
  • Class
  • Clinical significance
A

Lutheran antibodies:

  • Anti-Lu(a)
  • Cold
  • IgM
  • Insignificant
19
Q

What type of a reaction is a typical feature of Lutheran antibodies?

A

Mixed Field

20
Q

Where is HLA encoded?

A

Major Histocompatibility complex (MHC)

-chr6p

21
Q

MHC consists of what genes?

A

MHC

  • class I
  • class II
  • class III
22
Q

MHC class III genes encode for what?

A

Complement proteins

23
Q

What is also embedded in the MHC region? (3)

A
  • HFE gene
  • 21-hydroxylase gene (CAD)
  • TNF gene
24
Q

Class I genes encode for HLA class I antigens:

  • What cells
  • Distributed among what 3 loci
A
  • Surface of ALL cells

- HLA-A, HLA-B, HLA-C

25
Q

HLA class I genes encode a single polypeptide chain that is… (2)

A
  • Embedded as a transmembrane protein

- Noncovalently associated with a single molecule of a2-microglobulin

26
Q

Young RBCs express class I antigens but lose them as they age, what is the exception?

A

Bg (Bennet Godspeed) Antigens
-Strongly expressed on Mature RBCs

*Rarely cause HTR

27
Q

What are the major Bg antigens? (3)

A
  • Bga (HLA-B7)
  • Bgb (HLA-B17)
  • Bgc (HLA-A28/A2)
28
Q

T/F: Platelets have a generous amount of class I antigens.

A

True

29
Q

Class II genes encode class II antigens:

  • What cells (3)
  • What 3 loci
A
  • B-cells
  • Macrophages
  • Activated T-cells

HLA-DR
HLA-DP
HLA-DQ

30
Q

Class II genes encode what?

A

2 polypeptide chains (alpha/beta)

  • Each with 2 domains similar to the Ig light chains
  • Transmembrane protein
31
Q

T/F: Class II antigens are expressed on RBCs and Platelets.

A

FALSE

32
Q

HLA plays a small role in RBC compatibility but is pivotal in what situations? (3)

A
  • Platelet refractoriness
  • Solid organ compatilibity
  • Transfusion reactions (Febrile, TRALI, TAGVHD)
33
Q

Since each MHC comple is closely linked and inherited en bloc, each parental chromosome can be thought of as a haplotype. One hyplotype is inherited from each parent.

  • What is the chance that 2 siblings are HLA identical?
  • What is the chance if you have 2,3 siblings?
A

25%

  • 2 siblings - 45%
  • 3 siblings - 60%