Rh antigen Flashcards
What is the second autosomal linkage in humans?
Rh blood group and gene for elliptocytosis
What was the Fisher and Race theory?
3 closely linked genes Inherited has a haplotype Crossover was rare 1 gene = 1 antigen Did not explain all the low freq
What was the Weiner theory?
1 gene codes for 1 agglutinogen that has 3 factors
Each factor represented an antigen
Thought that R and r could code for unlimited factors
What is the ISBT number for Rh?
004
What was Tippetts theory?
Correctly described the 2 gene inheritance
1 RHD gene and 1 RHCE gene
What antigen does ISBT 4.6 code for?
f
What antigen does ISBT 4.7 code for?
Ce
What antigen does ISBT 4.8 codes for?
Cw
Most prevalent Rh phenotypes in whites?
R1 (42%) > r (37%) > R2 (14%) > Ro (4%)
68% of whites are C +
80% are c+
Most prevalent Rh phenotypes in Blacks?
Ro (44%) > r (26%) > R1 (17%) > R2 (11%)
27% are C+
98% are c+
Characteristics of Rh antibodies
IgG; rarely IgM
Do not activate complement
- Causes extravascular hemolysis
Enhanced by enzymes
Rh most to lease immunogenicity
D > c > E > C > e
What is anti-RH17?
RH17 = Hro (High freq)
antibody that reacts with all cells except D– and D**
Auto antibody is known as anti-pdl (partial deleted)
What is anti-RH29?
RH29 = total Rh
antibody that reacts with all cells except Rh null cells
Immune stimulated in Rh null
Some anti-RH29 reacts with Rh mod cells
auto antibody is known as anti-dl (total deleted)
What are anti-hr(s) and anti-hr(b)?
antibody reacts with all cells but can look anti-e reacting with stronger with e+ cells. Made by people with variant e. Seen in black population.
What is anti-LW?
Antibody reacts stronger with D+ cells than D- cells
- May need to enhance to show reactivity with D- cell
Negative with Rh null cells
Anti-LW reacts with all cords regardless of Rh type
DTT destroys
Seen as an auto antibody with no clinical significance
Cw
Found with multiple antibodies
Cw is found in 2% of whites
Goa
Goa (RH30) is present in DIVa partial D cells
present in 2% of blacks
V antigen is historically known as
ce^s
RH10
occurs in 30% of blacks
The VS antigen is historically known as
e^s
RH20
occurs in 32% of blacks
What is anti-Evans antibody?
Evans antigen is present on D** cells and not D– cells
What is anti-f?
f is present with c and e are in cis
present on Ro and r cells
Appears to be anti-c and -e, but only reacts to shared determinate on Ro and r cells
What is anti-G?
reacts with all cells carrying D or C or both
Transfuse D- C- blood
Differentiate in pregnant females for RHIG
What amino acid change makes G antigen?
Serine at position at 103 on RHD and RHCE gene
Anti-Ce
Also called RH7 or rhi
Similar to f, reacts with Ce+ cells in cis (R1 or r’)
Most anti-C is Ce in Rh positive people
Ce is present in 68% of White and 27% of blacks
- Most C+ will be e+
What disease show reduced expression of Rh antigens
Hereditary spherocytyosis Leukemias Myeloid metaplasia Myelofibrosis Polycythemia
What causes anemia in Rh null or Rh mod people
RHag is required for strong cytoskeleton of RBC
Integrity of membrane is compromised
Increased osmotic fragility - abn cation fluxes
Stomatocytes present
What chromosome is RHD and RHCE located on?
Chromosome 1
Characteristics of RHD and RHCE genes?
Both are 416 amino acids long with 10 exons
92-97% identical
genes run in opposite direction = “hair pin” formation and genetic recombination
What chromosome is RHAG located on?
Chromosome 6
What chromosome is LW located on?
Chromosome 19
What cells have the most D antigens present?
D–
D**
R2R2
What is the amino acid that determines C
Serine at 103
Ser103Pro
What is the amino acid that determines c
Proline at 103
Ser103Pro
What is the amino acid that determines E
Proline at 226
Pro226Ala
What is the amino acid that determines e
Alanine at 226
Pro226Ala
What is the Rh box?
RHD gene is flanked by 2 Rh boxes
Identity boxes
In whites, Deletion of D gene occurs in RH boxes
What is the structure of D antigen?
Pass membrane 12x Has a C and N terminus in cytoplasma of cell Non-glycosylated = Hydrophobic Non=phosphorlated More than 30 epitopes
What is X1r?
Normal RHAG gene
Makes precursor substance 2 from precursor 1
Must be present to make RH and LW antigens
What is Xor?
No RHAG gene
= Rh null regulator
No Rh antigens or LW antigens
What is Xqr?
Rh mod phenotype
RHAG makes a modified precursor substance 2. DCE and LW antigens are present but weak
What is rr (double bar)
Amorph Rh null
No DCE or LW antigens
RHAG is normal
D deletion with homozygous inactive RHCE genes
High protein Anti-D
Human source = Polyclonal
20-24% protein added to enhance IS of IgG antibody by bringing RBC’s close together
May see spontaneous agglutination with warm auto
IgM Saline anti-D
Human source
Expensive because hard to find donors with IgM Anti-D
Chemically modified anti-D
Human source
DTT breaks the disulfide bonds in the hinge region to allow IgG to crosslink 2 RBC’s
Monoclonal anti-D
Combonations of IgM and IgG
Can be monoclonal IgM with human IgG
or both monoclonal IgG and IgM