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RH Blood Group

RH Blood Group System

QuestionAnswer
Chromosome location and gene structure of the Rh locus Chromosome 1
consists of two closely linked genes: RHD (codes for D antigen) and RHCE (codes for C and E antigens) Rh Locus
Molecular difference between C/c antigen single amino acid difference: C(Serine) vs c (Proline) at osition 103
Molecular difference between E/e antigen single amino acid difference: E(Proline) vs e (Alanine) at position 226
Rh Antigens are determined by 3 pairs of closely linked genes (3 loci: D/d, C/c, E/d) inherited together as a haplotype Fisher-Race genetic theory
Postulates that 1 gene on each chromosome controls total Rh expression, producing a single agglutinogen containing multiple factors Wiener genetic theory
Two locus: RHD codes for RhD protein and RHCE codes for RhC and RhCE protein. RHAG on a separate loci is required for expression Molecular theory of Rh Genes
Wiener shorthand for DCe R1
Wiener shorthand for DcE R2
Wiener shorthand for Dce R0
Wiener shorthand for DCE Rz
Wiener shorthand for dce r
Wiener shorthand for dCe r'
Wiener shorthand for dcE r"
Wiener shorthand for dCE ry
Most common D-positive phenotype R1r (DCe/dce)
Most common D-negative phenotype rr (dce/dce)
Order of immunogenicity for Rh group antigens D > c > E > C > e
General characteristics of Rh antibodies IgG antibodies reacting at 37C/IAT, do not activate complement or cause extravascular destruction
Quantitative reduction in antigen: inheritance of an RHC gene that codes for reduced density of structurally normal D antigens on RBCs Genetic Weak D mechanism
Quantitative reduction in antigen activity: steric hindrance caused when the C gene is inherited in trans position to the D gene (Dce/dCe) Positional Effect Weak D mechanism
Qualitative change: RHD gene mutation causes structural alteration missing standard epitopes. Patient can produce anti-D against missing parts D Variant (Partial D) mechanism
True or False D variant is determined only by indirect antiglobulin testing True
D Variant patient as a blood donor: their blood is labeled as Rh - POSITIVE or Rh - NEGATIVE Rh-POSITIVE
D Variant patient as a blood recipient: their blood type is labeled as Rh - POSITIVE or Rh - NEGATIVE Rh-NEGATIVE
Complete absence of all Rh antigens (---/---); causes membrane abnormalities leading to stomatocytosis and compensated hemolytic anemia Rh Null phenotype
epitope expressed only when c and e genes are inherited on the same chromosome in the cis position (dce or Dce) f (ce) compound antigen
present on RBCs with C or D antigens, but has anti-C and anti-D activity, requires giving D-negative and C-negative blood G antigen
low-frequency variant Rh antigen usually associated with C; can be naturally occurring or immune-stimulated Cw antigen
Rh-associated glycoprotein resides on Chromosome 6 and is required for Rh antigen expression on the RBC membrane. RH AG
Capital R = D-positive; Lowercase r = D-negative. 1 or ' = C; 2 or'' = E; 0 or plain r = neither C nor E; z or y = C and E Fisher Race - Wiener conversion rules
Deletion/non-function at Cc and/or Ee loci. Displays exceptionally strong D expression but lacks C, c, E, e D- deletion (-D- or D--)
The variation in antibody reaction strength depending on whether an antigen is expressed in a homozygous (stronger) or heterozygous (weaker) state on the RBC surface. Dosage effect
The location of two genes on the same chromosome (e.g., c and e on dce) Cis Position
The location of two genes on opposite homologous chromosomes (e.g., D on one chromosome and C on the other in Dce/dCe). Trans Position
A two-stage lab technique used to detect cell-bound IgG antibodies in vitro after incubation, washing, and addition of anti-human globulin. Indirect Antiglobulin Test (IAT)
mechanism of Weak D is qualitative, require special clinical handling for transfusion recipients Partial D or D Variant
Most common unexpected Rh antibody in antibody screening anti-E
2nd most immunogenic; watch for concomitant anti-c when an R1R1 patient forms anti-E c antigen
Associated with $G$ antigen, Cw, and trans-position Weak D effect C antigen
High-frequency antigen (>98\%); most common specificity in warm autoantibodies (WAIHA) e antigen
Can be immune-stimulated or naturally occurring (often appearing as a weak IgG or IgM without clear prior transfusion/pregnancy exposure) Anti-E
Created by: MOrfCLS
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