Term
| Name the four main warm reacting systems |
|
Definition
| Kell, Duffy, Kidd, Lutheran (warm/cold) |
|
|
Term
| What are the antithetical pairs in the Kell blood group system? |
|
Definition
K (K1) & k (K2)
Kpa (K3) & Kpb (K4)
Jsa (K6) & Jsb |
|
|
Term
| Kell antibodies are produced in response to antigen exposure through _____ &______. |
|
Definition
| transfusion or pregnancy (HDN) |
|
|
Term
| Kell: Location and function. |
|
Definition
| Found only on RBC. It is present on fetal RBC. It is located on glycoprotein. The function is indeterminant but it's an integral part of the RBC membrane. |
|
|
Term
| Kell antigens are sensitive to treatment with what reagents? |
|
Definition
Sulfhydryl reagents
- 2-mercaptoethanol
- dithiothreitol
- 2-aminoethylisothiouronium bromide |
|
|
Term
|
Definition
Reduces disulfide bonds.
Kell glycoporteins possesses extensively folded disulfide bonded region. Treatment of RBCs with these reagents creates RBCs lacking kell antigens. |
|
|
Term
|
Definition
Strong. 2nd to D antigen in terms of eliciting an immune response in transfusions.
Other antigens in Kell system are less immunogenic. The antibodies are not common due to antigen frequency and immunogenicity. |
|
|
Term
| What are the high incidence genes of the Kell system? |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| Depressed reactivity , use albumin |
|
|
Term
| What's the most common Kell antibody? |
|
Definition
|
|
Term
| Comparison between antibody frequency in a white and black population. k, kpb, jsb |
|
Definition
| Anti-k and anti-kpbassociated with white population. anti-Jsb with blacks. They are rare. |
|
|
Term
| XK1 gene located on X chromosome codes for what antigen? |
|
Definition
|
|
Term
| Phenotypic Relationship between Kell and Kx. |
|
Definition
Inherited seperately.
RBCs with normal Kell phenotypes carry trace amount of Kx.
RBCs from Ko individual possess elevated Kx. |
|
|
Term
|
Definition
Lack of XK1 gene results in no Kx antigen. Absence of Kx antigen and weaked expression of Kell antigens results in McLeod phenotype.
Rare, but found in white males. |
|
|
Term
|
Definition
RBC abnormality.
Muscular and Neurologic defects
Increased CK. Chronic granulomatous disease. |
|
|
Term
| Chronic granulomatous disease |
|
Definition
associated with McLeod syndrome.
Function of phagocytic WBC is impaired. It can engulf but not kill microorganism. |
|
|
Term
| Ko or Kell null phenotype |
|
Definition
RBCs lack expression of all Kell antigens. Results from inheriting 2 recessive Ko genes.
Instead it expresses Kx antigen. |
|
|
Term
If Ko individual receives transfusion can stimulated to make anti___.
Significance. |
|
Definition
Anti-Ku (K5)
It is clinically significant since Ku antigen is present on all RBCs except Ko cells. |
|
|
Term
| Ku in relation with Kell antigens. |
|
Definition
|
|
Term
| Duffy antibody is found in serum of what kind of individual? |
|
Definition
| multiply transfusion individual or haemophiliacs. |
|
|
Term
|
Definition
Fya & Fyb (antithetical)
Fy3, Fy4, Fy5, Fy6 |
|
|
Term
| What 3 duffy antigens can be destroyed by papain or ficin? |
|
Definition
|
|
Term
| What 2 Duffy antigens are not expressed when the RBCs are Fy(a-b-) |
|
Definition
|
|
Term
| The 3 Duffy antigens that are resistant to ficin/papain treatment. |
|
Definition
|
|
Term
| Biochemistry of Duffy antigens. (chemical makeup, location , function) |
|
Definition
| GLycoprotein on RBC membrane that spans the lipid bilayer of the membrane several times. They are the receptors for proinflamatory chemokines.Chemiokines are involved in the activation of WBCs during migration across the endothelium. |
|
|
Term
| Fya & Fyb are ______ alleles |
|
Definition
Codominant alleles
EX: Fy(a+b+) |
|
|
Term
|
Definition
|
|
Term
|
Definition
| no identifiable Duffy antigen (an amorph) Black people |
|
|
Term
| Clinical signifiance of Duffy |
|
Definition
| Transfusions. Production is stimulated by exposure to antigens through pregnancy or transfusions. |
|
|
Term
| Frequency comparison : anti-Fya & anti Fyb |
|
Definition
| Anti fya is more common than anti fyb |
|
|
Term
| Fya & Fyb antigens + enzymes |
|
Definition
| Duffy is destroyed by enzymes. |
|
|
Term
| Duffy antigens and dosage. |
|
Definition
Yes it demonstrates dosage.
Ex: Fy(a+b-) or Fy(a-b+) |
|
|
Term
|
Definition
| Fy(a-b-) rbc are resistant to infection by Plasmodium knowlesi and P. vivaxi. Duffy antigens are receptors for attachment of the merozoite to RBCs. |
|
|
Term
|
Definition
Linked to extravascular hemolysis and delayed hemolytic transfusion reactions. Detection of Kidd alloantibodies sometimes difficult due to rise and quick fall of antibodies to undetectable levels.
Weak reactivity in vitro but capable of severe RBC destruction in vivo. |
|
|
Term
|
Definition
Jka, Jkb, Jk3 (present when Jka or Jkb is present)
Develop early in fetal life. |
|
|
Term
|
Definition
Jk(a-b-)
Resistant to lysis in 2M urea. Suggests that Kidd antigens function as urea transporters. |
|
|
Term
|
Definition
| Supressor gene of Kidd system. |
|
|
Term
| Jka & Jkb are ____ alleles |
|
Definition
|
|
Term
| Kidd antibodies + enzymes, LISS, PEG |
|
Definition
|
|
Term
| Kidd antibodies often appear with sera of individuals with _____ antibodies |
|
Definition
|
|
Term
| Kidd antibodies and dosage |
|
Definition
|
|
Term
| Similarities of DUffy, Kidd, and Kell. |
|
Definition
| IgG, RBC simulated, react at AHG phase of IAT, and clinically significant. |
|
|
Term
|
Definition
| Not very dangerous but it is immunogenic when multiple antibodies are present. |
|
|
Term
| Cold Reacting blood systems (4) |
|
Definition
| Lewis, I/i, P, M/N(S/s, U -warm) |
|
|
Term
| Lewis Blood Group characterstics and clinical signficance. |
|
Definition
Different from other blood group ag. It is manufactured by tissue cells & secreted into body fluids. It is found primarily in secretions and plasma. It is adsorbed onto RBC membrane. (not part)
Not clinically significant. |
|
|
Term
|
Definition
L-fucoslytransferase.
Adds a fucose to the subterminal sugar N-acetylglucosamine of type 1 precursor substance. It becomes Le(a) antigen and is adsorbed onto the RBCs. |
|
|
Term
|
Definition
| If H substance is in secretion, Le gene transfers the fucose to the H substance producing the Le(b) antigen. |
|
|
Term
| Lewis phenotype and Newborns |
|
Definition
| Reliable Le phenotype is not possible until about 6 years old. Le(a-b-) for Newborn. |
|
|
Term
| Antigen development of Lewis is dependeont on which 3 independent sets of genes? |
|
Definition
|
|
Term
Lewis antibodies frequencies.
-Le(a-b-)
-Le(a-b+)
-Le(a+b-) |
|
Definition
- Anti Lea or Leb can be found and it occurs almost exclusively in these individuals.
-Le(a-b+) do not make antibody
-rarely makes the antibody.
Important to note that the antibody does not require RBC stimulation. |
|
|
Term
|
Definition
|
|
Term
| Lewis can be challanging to ID because of.... |
|
Definition
Wide temperature range of reactivity.
Agglutination may be at IS, 37C or AHG.
Hemolysis may be seen in vitro because antibodies binds complement efficiently. |
|
|
Term
| I & i antigen relationship to A,B, H |
|
Definition
| exists on the same precursor chain. |
|
|
Term
|
Definition
| I is branched, i is linear |
|
|
Term
|
Definition
| plasma, RBC, body secretions |
|
|
Term
|
Definition
| i comes first before I due to structure. So it takes time to convert. |
|
|
Term
| I/i clinical significance |
|
Definition
| Clinically insignificant (IgM). Anti-I is usually an autoantibody. Alloanti I is rare. |
|
|
Term
|
Definition
|
|
Term
| Disease association with Anti-I/i |
|
Definition
| Strong autoanti-I, associated with Mycoplasma pneumoniae infections and cold hemagglutinin disease. Anti-i associated with infectious mononucleosis, lymphoproliferative disease, and occasional cold hemagglutinin disease. |
|
|
Term
|
Definition
|
|
Term
| P system relation with ABH |
|
Definition
structurally related, because it is formed by action of glycosyltransferases.
Expression of P system antigen originates from 2 indepndently inherited genes. P1 antigen is present in soluble form in secretions. |
|
|
Term
| Hydatid cyst fluid and P1 antigen |
|
Definition
|
|
Term
| Anti-P1 clinical significance |
|
Definition
Not clinically significant, but it can transfuse P1 positive cells if compatible at 37C and AHG.
Does not require RBC stimulation. |
|
|
Term
| Anti P clinical significance |
|
Definition
| rare, significant; associated with abortions. |
|
|
Term
|
Definition
or Anti-Tja
It may be made by individuals homozygous for p gene (null).
Significant. Requires blood from P null donor. |
|
|
Term
|
Definition
| associated with paroxysmal cold hemoglobinuria. IgG; called Donath landsteiner antibody. Biphasic hemolysin. May appear transietnly in children following viral infections & in adults with tertiary syphilis. Transfused cells must be warmed. |
|
|
Term
| Donath landsteiner antibody |
|
Definition
Auto-anti P
Appear transietly in children following viral infections and in adults with tertiary syphilis.
Biphasic hemolysin. |
|
|
Term
| Gene GYPA and gene GYPB codes |
|
Definition
GYPA - glycoprotein A (M or N)
GYPA - glycoprotein B (S or s)
Inherited as haplotype due to closeness. |
|
|
Term
|
Definition
|
|
Term
|
Definition
| exhibits dosage and sticks out form cell membrane. |
|
|
Term
|
Definition
| U antigen is located near RBC membrane and is always present when S or s is inherited. |
|
|
Term
| Anti-M clinical significance |
|
Definition
IgM/IgG
-IgM is not clinically significant
-IgG is significant. Dosage |
|
|
Term
| Anti-N clinical significance |
|
Definition
IgM rarely encountered. No significant.
N like antibody found more frequently in dialysis patients exposed to formaldehyde-sterilized dialyzer membranes. |
|
|
Term
| Anti-S,s,U clinical significance |
|
Definition
IgG significant.
Can cause HTR or HDN.
Anti-U is rare but should be considered if antibody to high incidence antigen is in the serum of a previously transfused black person.
U negative blood found only in black donors (<1%) |
|
|
Term
|
Definition
| Lua and Lub - codominant, antibodies are occasionally found, but most population is Lu(a-,b+) |
|
|
Term
|
Definition
results from inhertance of recessive amorph gene, Lu.
Inheritance of rare inhibitor gene, or recessive X linked supressor gene. |
|
|
Term
|
Definition
No clinical signifance for transfusion, mild HDN reported.
** mixed field agglutination pattern. IgM & IgG. May be present without stimulation. optimal in vitro reactions at room temperature. |
|
|
Term
|
Definition
| rare antibody due to high incidence antigen. IgG. Agglutination at AHG. some mixed field, associated with transfusion reactions and mild HDN. |
|
|
Term
| Clinically significant Blood group systems. |
|
Definition
| A& B, Rh; Kell, Kidd, Duffy, S,s,U |
|
|
Term
| Sometimes clinically significant |
|
Definition
|
|
Term
| Clinically significant if reactive at 37C |
|
Definition
| A1; H; Lea; Lutheran; M&N, P |
|
|
Term
| Usually clinically insignificant |
|
Definition
|
|
