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Rh blood group

system 2
Describe the causes and effects of:
Weak D.
partial D.
Rh null.
Predict the reactions of antibodies
to compound antigens with the
common Rh genotypes.
Weak D Antigen.
Single amino acid switch in
transmembrane region of the RHD
protein.
Reduced expression of all epitopes.
Do not produce allo-anti-D.
Can immunise RhD neg recipients.

RHD positive as RBC


recipients or donors.
Weak D position Ceppellini effect

Inheritance R1R0 R2r


of C in trans
to D, weakens
expression of
D antigen.
antigen

RHD

R0r R2Ro R1r R1R2


Partial D phenotype.
Missense mutation in RHD leads to
partial expression or absence of RBC
some epitopes.
Can form anti-D.
Can immunise RHD neg
individuals.
RHD neg as recipients RBC
RhD positive as donors.
Rhnull phenotype
RBCs lack all Rh antigens.
Have defective RBC membrane:
altered RBC shape, osmotic fragility,
lifespan.
Can produce anti-RH29 if immunised
by transfusion.
S, s, U, and LW antigens are also
affected.
1961, Vos et al
Amorphic Rhnull
Homozygous inheritance of
inactivating mutation on CE
locus in an RHD neg individual.
R1- R2-
DCe DcE
DCed-- DcEd--

DCeDcE DCed-- d--DcE d-- d--


DCcEe DCe DcE ---/---

R1R2 R1- -R2 ---/---


Rhnull Regulator type
Mutations in
RHAG
Reduced amounts Total absence
of RHAG of RHAG

Reduced Rh Rh antigens
antigen expression not expressed

Rhmod Rhnull
Compound (cis) antigens
Additional antigen present when
genes are inherited in cis.
Cis antigen Haplotype
ce (RH6) R0 : r
Ce (RH7) R1 : r
cE (RH27) R2 : r
CE (RH22) RZ : ry

Antibodies Rare.
Anti-f (ce) is more common.
Marked dosage effects.
Inseparable.
Compound (cis) antibodies
Used to determine inherited
genotypes or haplotypes.
Cells tested with anti-
D C E c e Phenotype
+ + + + + DCcEe

Possible Genotypes
DCe/DcE Dce/dCE DCE/dce
R1R2 R0ry Rzr
Compound (cis) antibodies

Anti-Ce Anti-cE Anti-CE Anti-ce


R 1R 2 3 3 0 0

R0RZ 0 0 3 3

R0ry 0 0 3 3

R2r 0 4 0 0

R 1R 1 4 0 0 0

rr 0 4 0 0

rr 3 0 0 3

rr 0 0 0 4
G antigen (RH12)

Mostly found on RBCs with D


or C antigen.
rare D-C-G+ and D+C+G-
Common amino acid on both D
and C antigens. Haplotype
Patients who form anti-C R0 : r
often form anti-G as well. R1 : r
Inseparable anti-CD. R2 : r
RZ : r y
Anti-G (-RH12)

R 1R 2 4 rr patients transfused with rr donor


R 2R 2 4 blood often form anti-C + anti-G
R 1R 1 4

R 0r 3
rr patients transfused with R0r donor
rr 3
blood often form anti-D + anti-G
rr 3

rr 0

rr 0 Mimicks anti-C + anti-D


rr 0 Inseparable
rr 3

rr 0
Adsorption
C+

Anti-D + anti-G mixture


D-
C+
D-

Y
can be separated by C+
D-
Y Y Y
adsorption. Y Y
Y Y
Anti-D Y
Y C+

C positive D negative Anti-G


D-

RBCs incubated with


Patients serum. C+
Y
Y D-
C+
D-

Anti-G adsorbs onto C C+


Y
Y
Y
D-

positive cells leaving Y C+


D-

Y
anti-D in the serum Y Y
Y
Adsorption
Anti-C + anti-G mixture
C-
D+
C-
D+

can be separated by Y
Y Y Y
C-
D+

adsorption. Y Y
Y Y
Anti-C Y
D positive C negative Y
Anti-G
C-
D+

RBCs incubated with


Patients serum. Y
C-

Y D+
C-
D+

Anti-G adsorbs onto D C-


Y
Y
Y
D+

positive cells leaving Y C-


D+

Y
anti-C in the serum Y Y
Y
LW
1963 - RH and LW different Blood
Groups (Levine, Cellano et.al)
Chromosome 19p13.3
Similar serologic properties but not
genetically related.
Alleles are LWa, LWb, and LW
(amorph).
LW
D positive RBCs from adults
express LW more strongly than D
negative red cells
anti-LW made by LW(a-b-)
people can mimic anti-D.
all RHnull individuals are LW(a-b-),
but not all LW(a-b-) are RHnull
Further reading
Daniels G, Bromilow I, Essential guide to Blood Groups,
Wiley-Blackwell, Oxford.
Overfield J, Dawson D, Hamer D. Transfusion Science
2nd Ed 2008, Scion
Blainey and Howard, Basic and Applied Concepts of
Immunohematology Ch 5.2nd Ed.MOSBY Elsevier.
Missouri
Reid ME and Lomas-Francis C, The Blood Group
Antigens FactsBook, Academic Press Limited, London,
1st Edition, 1997
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