RH BLOOD GROUP SYSTEM Flashcards

(33 cards)

1
Q

Antigens of the Rh system

_____- refer only to presence or absence of the Rh antigen D on the red blood cell

●“Rh pos” and “Rh neg” are old terms, although blood products still labeled as such

●Early name “Rho” less frequently used

● Four additional antigens:______

●Named by Fisher for next letters of alphabet according to precedent set by naming A and B blood groups

●Major alleles are C/c and E/e

A

“D positive” and “D negative”

C, c, E, e

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3
Q

________-, after A and B, is the most important RBC antigen in transfusion practice

●Individuals who lack D antigen DO NOT have ____

  • produced through exposure to D antigen through transfusion or pregnancy

●Immunogenicity of D greater than that of all other RBC antigens studied

● Has been reported that >80% of D neg individuals who receive single unit of D pos blood can be expected to develop immune anti-D

____- is routinely performed so D neg will be transfused with D neg

A

D antigen

anti-D

Testing for D

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5
Q

Rh system IDENTIFIED by ____(2) in ___(year)

●Immunized animals to Rhesus macaque monkey RBCs
●Antibody agglutinated 100% of Rhesus and 85% of human RBCs
●Reactivity paralleled reactivity of sera in women who delivered infant suffering from hemolytic disease
●Later antigen detected by rhesus antibody and human antibody established to be dissimilar but system already named

A

Landsteiner and Wiener

1940

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6
Q

• Suggested that antigens are determined by 3 pairs of genes which occupy closely linked loci

• Each gene complex carries (3)

• Each gene (except d, which is an amorph) causes production of an ___

A

Fisher - race

D or its absence (d), C or c, E or e

antigen

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7
Q

MANY variations and combinations of the 5 principle genes and their products, antigens, have been recognized

_____- account for majority of unexpected antibodies encountered

_____- stimulated as a result of transfusion or pregnancy, they are immune

A

Rh antigens and corresponding antibodies

Rh antibodies

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8
Q

Key observation of ____(2) in ____(year) that the delivery of stillborn fetus and adverse reaction in mothers to blood transfusion from father were related

●Syndrome in fetus is now referred to as _______

  • Syndrome had complicated pregnancies for decades causing severe jaundice and fetal death.

●Erythroblastosis fetalis (HDN) linked with Anti-Rh by Levine in 1941

A

Levine and Stetson

1939

Hemolytic disease of the fetus and newborn (HDFN)

“Erythroblastosis fetalis”

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9
Q

• Three loci carry the Rh genes are so closely linked that they never separate but are passed from generation to generation as a unit or gene complex

A
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10
Q

Below an offspring of the
Dce/dce individual will inherit EITHER _____ from the parent,
never dCe as this would indicate crossing over which does not occur in Rh system in man.

A

Dce or dce

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11
Q

With the exception of __ each allelic gene controls presence of respective antigen on RBC.

____- would cause production of the D, C and e antigens on the red cells.

● If the same gene complex were on both paired chromsomes (DCe/DCe) then only ______ would be present on the cells.

● If one chromsome carried DCe and the other was DcE this would cause _____ antigens to be present on red blood cells.

● Each antigen except __ is recognizable by testing red cells with specific antiserum.

A

d

The gene complex DCe

D, C and e

D, C, c, E and e

d

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12
Q
  • Postulated that TWO genes, one on each chromosome pair, controls the entire express of Rh system.

● Each gene produces a structure on the red cell called an ______

● Eight (8) major alleles (agglutinogens): _____

● Each agglutinogen has 3 factors (antigens or epitopes)

●The three factors are the antigens expressed on the cell.

●For example the agglutinogen R0= Rh0 (D), hr’ (c), hr’ (e)

● Each agglutinogen can be identified by its parts or factors that react with specific antibodies (antiserums).

A

Wiener

agglutinogen (antigen).

R0, R1, R2, Rz, r, r’, r” and ry

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13
Q

The two theories are the basis for the two notations currently used for the Rh system.

● Immunohematologists use combinations of both systems when recording most probable genotypes.

● You MUST be able to convert a Fisher-Race notation into Wiener shorthand, i.e., Dce (Fisher-Race) is written ___

● Given an individual’s phenotype you MUST determine all probable genotypes and write them in both Fisher-Race and Wiener notations.

__ is the most common D positive genotype.

___ is the most common D negative genotype.

A

R0

R1r

rr

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14
Q

In ___ proposed a nomenclature based ONLY on serologic (agglutination) reactions.

● Antigens are numbered in the order of their discovery and recognition as belonging to the Rh system.

● No genetic assumptions made

● The ___ of a given cell is expressed by the base symbol of “Rh” followed by a colon and a list of the numbers of the specific antisera used.

● If listed alone, the Antigen is present (Rh:1 = ___)

● If listed with a __, antigen is not present (Rh:1, -2, 3 = DcE)

● If not listed, the antigen status was not determined

● Adapts well to computer entry

A

1962

phenotype

D Ag

“-”

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15
Q

__ is the most important blood group system after ABO in transfusion medicine

• One of the most complex of all RBC blood group systems with more than 50 different Rh antigens

The genetics, nomenclature and antigenic interactions are unsettled

A

Rh

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16
Q

____- International organization created to standardize blood group system nomenclature.

• Assigned 6 digit number for each antigen..

◦ First 3 numbers indicate the blood group system,
eg., 004 =

◦ Last 3 numbers indicates the specific antigen,
eg., 004001 =

• For recording of phenotypes, the system adopts the Rosenfield approach

A

ISBT international society of blood transfusion

Rh

D antigen

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17
Q

_____ is the result of the reaction between the red cells and antisera

_____ is the genetic makeup and can be predicted using the phenotype and by considering the race of an individual

• Only family studies can determine the true genotype

A

phenotype

genotype

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18
Q

Phenotyping and genotyping

Five reagent antisera available.

◦ Only ___ required for routine testing.

◦ Other typing sera used for typing rbcs to resolve antibody problems or conduct family studies.

• Agglutination reactions (positive and negative) will represent the ____

• No anti-d since d is an ____.

• Use _______ to determine most probable genotype.

A

anti-D

phenotype

amorph

statistical probabibility

19
Q

Rh phenotyping
Uses (4)

• Protocol
◦ Mix unknown RBCs with Rh antisera
◦ Agglutination indicates presence of antigen on cell and determines phenotype.
◦ Use published frequencies and subject information to determine genotype.

A

Parentage testing

◦ Predicting hemolytic disease of the fetus and newborn (HDFN)

◦ Confirmation of Rh antibody specificity

Locating compatible blood for recipients with Rh antibodies.

20
Q

Phenotyping and genotyping

Molecular testing becoming more popular:

◦ Cannot use ___ on recently transfused individuals, molecular testing can differentiate.

◦ Anti-sera not available for some antigens, molecular testing being developed for all blood group genes.

◦ D zygosity can be determined.

◦ Fetal genotyping for D can be done on ____

◦ Monoclonal reagents from different manufacturers react differently with variant D antigens, molecular test specific.

____ continue to be the “gold standard” but this will change in the future.

A

anti-sera

fetal DNA present in maternal plasma.

Typing sera

21
Q

____ - Refer to textbook.

____ are listed as “presumptive” or “most probable”.

• Genotypes will vary in frequency in different racial groups.

A

Genotype frequencies

Genotypes

22
Q

Not all D positive cells react equally well with anti-D.

• RBCs not immediately agglutinated by anti-D must be tested for ____.

◦ Incubate cells with anti-D at ___, coating of D antigens will occur if present.

◦ Wash X3 add AHG

◦ AHG will bind to ___ coating cells if present.

◦ If negative, individual is D negative

◦ If positive, individual is D positive

A

weak D

37C

anti-D

23
Q

3 mechanism of Weak D?

• Results in differences from normal D expression ◦

____- (inherited weak D or position effects)

____- (mosaic D; could produce Anti-D)

A

Genetic
• Position effect
• Mosaic

Quantitative

Qualitative

24
Q

Two systems of nomenclature developed prior to advances in molecular genetics

• Reflect serologic observations and inheritance theories based on family studies

• Because these are used interchangeably it is necessary to understand the theories well enough to translate from one to the other

• Two additional systems developed so universal language available for use with computers

25
Q

The order of loci on the gene appears to be ___ but many authors prefer to use “CDE” to follow alphabet

• Inherited from parents in linked fashion as _,,

• The gene __ is assumed to be present when D is absent

A

“DCE”

haplotypes

d

26
Q

• C trans - position effect; • The D gene is in trans to the C gene, eg., C and D are on OPPOSITE sides: Dce/dCe • C and D antigen arrangement causes steric hindrance which results in weakening or suppression of D expression.

27
C in trans position to D: D c e / d C e C in cis position to D: D C e / d c e Weak D NO weak D
28
29
If the patient is transfused with D positive red cells, they may develop an anti-D alloantibody* to the part of the antigen (epitope) that is missing
30
• Donors ◦ Labeled as D positive ◦ Weak D substantially less immunogenic than normal D ◦ Weak D has caused severe HTR in patient with anti-D • Patients ◦ If weak D due to partial D can make antibody to portion they lack. ◦ If weak D due to suppression or genetic expression theoretically could give D positive ◦ Standard practice to transfuse with D negative • Weak D testing on donors by transfusion service not required. • Weak D testing on patients not required except in certain situations.
31
___ refer to genes ____ refer to the agglutinogen (complex of antigens) • For example, the Rh1gene codes for the Rh1 agglutinogen made of D, C, e ◦ Usually, this can be written in shorthand, leaving out the “h” ◦ DCe is written as ___
Superscripts (Rh1) Subscripts (Rh1) R1
32
Genes that code for C or D also code for G ◦ G almost invariably present on RBCs possessing C or D ◦ Anti-G mimics anti-C and anti-D. ◦ Anti-G activity cannot be separated into anti-C and anti-D.
33
Very rare • Individuals inherit Rh gene complex lacking alleles. • May be at Ee or Cc • Must be homozygous for rare deletion to be detected. • No reaction when RBCs are tested with anti-E, anti-e, anti-C or anti-c • Requires transfusion of other D-deletion red cells, because these individuals may produce antibodies with single or separate specificities. • Written as D- - or -D-
34
• Red cells have no Rh antigen sites • Genotype written ---/--• The lack of antigens causes the red cell membrane to appear abnormal leading to: ◦ Stomatocytosis ◦ Hemolytic anemia • 2 Rh null phenotypes: ◦ Regulator type – gene inherited, but not expressed ◦ Amorph type – RHDgene is absent, no expression of RHCEgene • Complex antibodies may be produced requiring use of rare, autologous or compatible blood from siblings.
35
R  D r  no D 1 and ‘  C 2 and “  E Example: DcE  R2 r”  dcE