Lutheran Flashcards

1
Q

What are the Lu first?

A
  • 1st autosomal linkage discovered in humans was Se and Lu
  • 1st crossing over
  • 1st unlinked dominate suppressor gene
  • 1st x-linked dominate suppressor gene
  • 1st blood group with 3 different backgrounds for negative phenotype
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2
Q

How many antigens are in Lu system?

A
20 antigens
Lua/Lub
Lu6/Lu9     High/Low
Lu8/Lu14    High/Low
Lu18/Lu19 = Aua/Aub
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3
Q

What is frequency of Lua and Lub?

A

Lua 8% are positive
Lub is high freq
Lub negative 0.2% of most popuations
Lu3 is present when either Lua or Lub is present

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4
Q

What are the characteristics of Lu antigens?

A

Weak on cord cells; mild or no HDFN
Present on other tissues but not plasma, PLT’s, or WBC
Fragile when stored
Resistant to papain and ficin
Destroyed by trypsin, Chymotrypsin, Pronase AET, and DTT

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5
Q

How does DTT/AET affect Lu antigens?

A

DTT and AET destroy disulfide bond IgSF that carry LU antigens

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6
Q

How many Lu antigens are the RBC membrane?

A

Number of antigens vary depending on phenotype and person. may seen MF agglutination.
Lu(a+b-) and In(Lu) only has 500 antigen sites
Lub is weakend with Lua is present

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7
Q

What are the characteristics of anti-Lua?

A

IgG, IgM, IgA, rarely binds complement
May be naturally occuring IgM
may be reactive at lower temps (18C)
Often seen MF reaction
Moslty benign antibody; rare cause of HTR
HDFN is no or milk due Lu antigen on placenta

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8
Q

What are the characteristics of anti-Lub?

A

More clinically significant that anti-Lua
mostly IgG ; rarely C’
Can cause mild HTR with jaundice and slow clearance of RBC’s
Mild HDFN

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9
Q

What is Anti-Lu3

A

IgG; rarely binds C’
Reacts with all Lua or Lub positive cells
Made by LuLu recessive people = Lu(a-b-)

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10
Q

How do identify anti-Lub?

A

DTT destroys (also Detroys Kell, JMH, DO, and KN)

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11
Q

What are the characteristics of Lu 4-20 antigens?

A

NOt fully developed at birth
Resistant to ficin and papain
Sensitive to DTT and AET
Relatively few reports of each antibody specificity

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12
Q

What is the LuLu phenotype?

A

Recessive inheritance of 2 Lu null gene
Very rare
No Lu antigens = make anti-Lu3
Red cells will not adsorb/elute Lu antibodies

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13
Q

What is the In(Lu) dominate inhibitor?

A

In(Lu) is a DOMINATE gene located at another locus that inhibits Lu antigen expression
Caused by mutation in KLF1 gene
May see abnormal RBC morph = acantocytes
Can absorb/elute Lu antibody = small amt of antigen
Homozygous inheritance is not compatible with life

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14
Q

What other antigens are weakend in the In(Lu) phenotype?

A

P1, i, IN (CD44), AnWj, Knops, Csa, MER2
Think of family studies if parents are typing as P1 negative and child types as P1 positive. Its because parents P1 was suppressed. Child inherited normal Lu and P1 gene and expressed both.

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15
Q

What is the Lu(mod) x linked inhibitor?

A

Mutation in GATA-1 an x linked transcription factor
Causes Lu(a-b-) phenotype but Lub can be absorbed and eluted off
Found in Males
RBC have weak P and I but increased i

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16
Q

What chromosome is LU located on?

A

Chromosome 19

17
Q

What is the product of LU gene?

A

Lutheran glycoprotein or B-CAM

18
Q

What is the structure of Lu antigens

A

Single pass glycoprotein that belongs to IgSF
5 immunoglobulin superfamily (IgSF) connected by disufide bonds
Contains repeating domains of Variable and constant regions

19
Q

How are the low frequency antigens formed?

A

Original Lu gene contained the high frequency antigens and SNP gave rise to the low freq. antigens
Lua, Lu9, Lu14, Lu18

20
Q

What is the function of the Lu antigen?

A

Adhesion molecule of IgSF that transfers mature RBC;s from BM to peripheral circulation

21
Q

What disease is associated with Lu antigens?

A

B-CAM is increased in some malignant tumors and cells
May mediate adhesion of sickles cells to endothelium
May be associated polycthemia vera