UNCOMMON BLOOD GROUPS P3 Flashcards
1
Q
In 2014, the blood grpup number 035 was assigned to the CD59 system based
on a CD59-deficient child who formed an alloantibody with CD59 specificity
A
The CD59 (035) System
2
Q
(CD59 ANTIGEN)
- The gene CD59 is located on ________ at position 11p13 and has only one antigen, CD59.1
- _____ is a GPI-linked complement-regulatory glycoprotein also known as the __________
- CD59 plays a key role in protecting against complement-regulated hemolysis by binding to C8 and C9 thus interfering with the formation of the membrane- attach complex (MAC). _____ is acquired hemolytic anemia caused by a mutation in the GPI-linker gene.
- _________ individuals have beem identified among Japanese,
North Africans, Jews, and Turks.
A
chromosome 11 ;
CD59 ; membrane inhibitor lysis (MIRL) ;
PNH ;
Congenital CD59-deficient
3
Q
(CD59 ANTIBODIES)
- _______ was IgG, which shoed increased reactivity with enzyme-treated RBCs and was nonreactive with DTT-treated RBCs.
- Patients with CD59 deficiency show PNH-like symtoms, including hemolysis
and strokes, but also neutropathy.
A
Anti-CD59.1
4
Q
- Anti-Ata was first described in 1967 in the serum of a black woman named Mrs. Augustine.
- The ISBT blood group system status was assigned in 2015 with a symbol of AUG.
A
The Augustine (036) System
5
Q
(AUG ANTIGEN)
- ____ is a high-prevalence antigen, and all At(a-) individuals have been black.
- Two antigens are ____ AND ____(Ata)
- The antigen defined by the antibody produced with the null phenotype is
AUG1. - The AUG gene, SLC29A1, located on ______ at position 6p21.1, encodes
the ________ - The antigens are fully developed at birth and are resistant to treatment with ficin and papain, DTT, and glycine-acid EDTA.
A
Ata ;
AUG1 and AUG2 ;
chromosome 6 ; equilibrative nucleoside transporter 2 (ENT2) ;
6
Q
(ATA ANTIBODIES)
- Anti-Ata is usually IgG, reactive in the antiglobulin phase testing, and has
caused severe HTRs and one reported mild case of HDFN. - A new low-prevalence antigen has been provissionally assigned _____; the
corresponding antibody caused severe HDFN.
A
AUG3
7
Q
- Collections are antigens that have a biochemical, serologic, or genetic
relationship but do not meet the criteria for a system. - Most the antigens in the blood group collection are either high or low
prevalence. - Antigens classified as a collection are assigned a 200 number.
A
ISBT Blood Group Collections
8
Q
- formally referred to as Cost-Sterling, was established in 1988.
- It was named after the two patients (Copeland and Sterling) who made anti-Csa.
A
Cost Collection 205
9
Q
COST COLLECTION 205 ANTIGEN
- Five antigens formally in the Cost Collection have been placed into the Knops system once it was established that they are carried on ________
- The Cost Collection currently consists of two antithetical antigens, ____ and ___
- The ___ antigen is a high-prevalence antigen with a frequency of 95% in the black population and greater than 98% in most population.
- The ___ antigen is a polymorphic antigen with a frequency of 34% in most populations.
- Both the Csa and Csb antigens demonstrate a resistance enzyme treatment aand
varying effects to treatment with DTT.
A
complement receptor 1 (CR1). ;
Csa and Csb. ;
Csa ;
Csb ;
10
Q
Antibodies
* The ________ antibodies are IgG reacting at antiglobulin phase of testing, and have not been found to be clinically significant.
* The antibodies are difficult to identify due to the varying RBC expression from person to person.
A
Cost Collection
11
Q
- Was first assigned in 1990 and consistefd of two antigens, I and i.
- In 2002, the I antigen was promoted to a blood group system 027, leaving i the
sole antigen in the collection.
A
Ii Collection 207
12
Q
Ii Antigen
- The i antigen occurs on unbranched carbohydrate chains and is the precursor for the I antigen.
- Along with RBCs, the I and i antigens are found on most human cells and on soluble glycoproteins
in the body fluids. - The i antigen is strongly expressed on cord cells with only trace amounts on adult RBCs
- is resistant to both enzyme and chemical treatments.
- may increase in expression during hemopoietic stress due to rapid production of RBCs.
A
Ii Collection 207
13
Q
Ii Antibodies
- Anti-i is usually IgM reacting at room temperature or 4 Degrees Celsius, some may bind
complement. - is not known to cause transfusion reactions and rarely causes HDFN.
- Autoanti-i is cold agglutinin associated with infectious monocleosis and some
lymphoproliferative disorders that occasionally can cause hemolysis.
A
Ii Collection 207
14
Q
was established as a collection in 1990 with three antigens.
A
Er Collection 208
15
Q
Er Collection 208 Antigen
- Two of the antigens are high-prevalence antigens, _____ and ____.
- Third antigen is a low-prevalence antigen, ____.
- The Er antigens are resistant to enzyme and DTT treatment, but sensitive to glycine acid/EDTA
treatment.
A
Era and Er3 ;
Erb
16
Q
Er Collection 208 Antibodies
- _____ is produced by the presumed null phenotype Er(a-b-).
- The antibodies are IgG reacting at the antiglobulin phase of testing and do not fix complement.
- Limited data exist on the clinical significance of Er antibodies.
- A patient with anti-Er3 showed mild hemolysis following transfusion of an incompatible unit.
- Monocyte-monolayer assays (MMA) suggest anti-Era is not clinically significant, whereas anti-Er3
had potential significance. - The Er antigens are expressed on cord cells, and anti-Era and -Erb have caused a positive DAT in
the newborn but no clinically significant HDFN.
A
Anti-Er3
17
Q
- The Bloboside collection currently consists of one high-prevalence antigen,
LKE. - The GLOB collection and how it relates to the P1PK (003) and Globoside (028)
blood group systems.
A
Globoside Collection 209
18
Q
- Consist of two antigens, Lec and Led, which are glycosphingolipids adsorbed onto RBCS.
- Lec and Led are precursors to the Lewis antigens and demonstrate increased expresion in
Le(a-b-) individuals. - The antigen names are misleading because neither Lec nor Led are produced by a Lewis
gene transferase.
A
Collection 210 (Unnamed)
19
Q
Collection 210 (Unnamed) Antigen
- ___ is a low-prevalence antigen occuring in 1% of most populations.
- ____ is a polymorphic antigen occuring in 6% of most populations.
A
Lec ;
Led
20
Q
- ______ agglutinates Le(a-b-) RBCs from nonsecretors.
- both humans and goats are known to make anti-Lec.
- _____ agglutinates Le(a-b-) RBC form secretors, but not from Le(a-b-)
- The discovery of anti-Led was the result of injecting goats with saliva from a Le(a-b+) individuals.
A
Anti-Lec ;
Anti- Led
21
Q
MN CHO Collection 213 Antigen:
- The MN CHO collection currently consists of six polymorphic antigens: __________________
- The antigens are associated with the M or N antigen in the MNS (002) system and are expressed on ____ with altered levels of sialic acid or GlcNAc.
- All are antigens are sensitive to ficin/papain, with some demonstrating resistance to a-chymotrypsin.
- The Hu antigen (213001) was formerly called ______ after the donor of the RBCs used to
immunize rabbits in 1934.
A
Hu,M1, Tm, Can, Sext, and Sj. ;
GPA ;
Hunter
22
Q
MN CHO Collection 213 Antigen:
- The Hu antigen is predominantly an altered GPAm with a strong link to the Sext antigen, which is also in the MN
CHO collection. - Enzyme treatment shows sensitive to ficinpapain and trypsin and resistance to a-chymotrypsin.
- The _______ (213002) was reported in 1960 and is predominantly an altered GPAm.
- Enzyme treatment of the M1 antigen shows sensitivity ficin/papain and trypsin and resistance to
a-chymotrypsin.
A
M antigen ;
23
Q
- In 1965, the _____ antigen was renamed Tm (213003).
- The logic behind the Tm naming was that “T” was next in line after the S and s, and since the “T” was already
associated with polyagglutination, the letter “m” was included due to the association with the MN system. - Enzyme treatment of the Tm antigen shows sensitivity to ficin/papain and trypsin and resistance to
a-chymotrypsin.
A
Sheerin
24
Q
- The ____ antigen (213004) was reported in 1979 and named after the first antigen-positive proband, Canner.
- The Can antigen is predominantly an altered GPAm that is sensitive to ficin/papain and trypsin and resistant to
a-chymotrypsin.
A
can
25
Q
- The ___ antigen (213005)was reported in 1974 and named after the individual who produced the antibody.
- The Sext antigen is predominantly an altered GPAn with a strong link to the Hu antigen. Sensitive to ficin/papain,
trypsin and salidase.
A
Sext
26
Q
- The ___ antigen (213006) was reported in 1968 when an anti-Tm serum was shown to have an additional
specificity. - The antigen is predominantly associated with GPAn and sensitive to ficin/papain
A
Sj
27
Q
(MN CHO Collection 213 Antibodies)
- All antibodies to the MN CHO collection react optimally at _______
- Antibodies to the Hu antigen have only been demonstrated in immunized rabbits.
- The majority of anti-M1 are made by M- individuals, but a few cases of anti-M1 have been found in M+N+ individuals.
- The separation of anti-M and anti-M1 by differential Adsorption has not been
successful.
A
room temperature
28
Q
- Antigens in the 700 series of low-prevalence antigens represent those with a
prevalence of less than 1% of most random populations. - The low-prevalence antigen and antibody discovery is usually unexpected.
Some examples of how the low-prevalence antigen/antibodies were detected are
(1) an unknown maternal antibody causing HDFN,
(2) an unexplained incompatible crossmatch, and
(3) unexplained positive reactivity with commercial human source antisera
containing an unknown low-prevalence antibody. - Low-prevalence antibodies are commonly found in serum that contains
multiple antibodies, especially antibodies to other low-prevalence antigens. - A case of severe HDFN that required three intrauterine transfusions was seen in
a neonate due to the presence of anti-HJK. - HDFN caused by anti-Kga and -REIT required exchange transfusion.
- Other cases of HDFN ranged in severity resulting in a positive DAT,
phototherapy, and/or transfuion.
A
ISBT 700 Series
29
Q
- Antigens in the 901 series of high-prevalence antigens represent those with a
prevalence of more than 90% of most random populations. - Six antigens currently make up the 901 series of the ISBT.
- The difficulty in identifying and finding compatible units makes antibodies to
the high-prevalence antigens a potential transfusion risk.
A
ISBT 901 Series
30
Q
- First reported in 1987 and named after the first identified antigen-negative
proband. - Six probands with the negative phenotype have been identified.
- antigen is expressed on cord cells and is resistant to all enzyme and chemical
treatments. - Anti-Emm has been identified to be both IgG and IgM reacting at Both 4 Degrees
Celsius and antiglobulin phase of testing with a higher occurence of IgG antibodies. - Some Have been shown to bund complement.
A
Emm Antigen
31
Q
- In 1982, an alloantibody to an antigen called Anton was identified and followed
by the identification in 1983 of an autoantibody to an antigen call Wj. - In 1985, it was shown that they were the same antigen and renamed AnWj.
- Is not expressed on cord cells and has varying expression in adults.
- Antigen is resistant to ficin/papain, trypsin, and a-chymotrypsin, and is variable
with DTT. - Haemophilus influenza uses the AnWj antigen as a receptor to enter RBCs.
- Anti-AnWj is an IgG antibody demonstrating reactivity at antiglobulin phase of
testing; - most anti-AnWj are autoantibodies due to a trasnfusion suppression of the
AnWj antigen.
A
AnWj Antigen
32
Q
- is a high-prevalence carbohydrate amtigem named for Sid, who was the head of the maintenance department at the Lister Institute in London.
- The soluble form of Sds is Tamm-Horsfall glycoprotein found in urine.
- The antigen is not expressed on RBCs of newborns but is in their saliva, urine,
and meconium. - The Sda antigen is resistant to treatment with ficin, papain, DTT, and glycine-acid EDTA.
- Anti-Sda can naturally occur in the sera of individuals who are Sd(a-).
- Usually an IgM agglutinin that is reactive at room temperature, but it can be detected in the indirect antiglobulin test and does not react with cord RBCs.
- Anti-Sda is generally considered clinically insignificant for transfusion, though there are two reports of transfusion reaction associated with the transfusion of
Sd(a++) RBCs.
A
Sda Antigen
33
Q
- The High-prevalence PEL antigen was first identified in 1980 and given the
name PEL in 1966 after the first negative proband. - The PEL-negative phenotype has been identified in two French-Canadian
families. - Antigen is expressed ocn cord cells and is resistant to both enzyme and
chemical treatment. - Anti-PEL are presumed to be IgG reacting at the antiglobulin phase of testing.
- No signs of HDFN were noted for the baby of the original PEL-negative proband.
A
PEL Antigen
34
Q
- In 1996, the high-prevalence ABTI antigen was reported with the detection of anti-ABTI in three multiparous women of an
inbred Israeli-Arab family. - The ABTI- phenotype was also reported in one Bavarian and one German individual.
- The expression on cord RBCs was presumed to be the mechanism of immunization.
- The ABTI antigen is resistant to enzymes and chemical treatment.
- Anti-ABTI are IgG reacting at the antiglobulin phase of testing.
- Anti-ABTI has not demonstrated evidence of HDFN.
A
ABTI Antigen
35
Q
- The high-prevalence antigen MAM was first reported in 1993 and assigned to
the 901 series in 1999. - 4 MAM- proband have been reported.
- All the probands are thought to have formed anti-MAM through exposure
during pregnancy because none of the probands had a history of transfusion. - MAM antigen is expressed on cord cells, lymphocytes, granulocytes,
monocytes, and probably platelets. - Antigen is resistant to both enzyme and chemical treatment.
- It is thought that anti-MAM may also cause neonatal thrombocytopenia.
- Anti-MAM are IgG, reacting at the antiglobulin phase of testing.
A
MAM Antigen
36
Q
- HLA class I antigens (HLA-A,-B, and -C) are present on all nucleated cells.
- HLA antigens are not considered a blood group antigen.
- The name “Bg” (Bga, Bgb, and Bgc) was given to HLA class I antigens that are
detectable on mature RBCs. - Bga = HLA-B7
- Bgb = HLA-B17
- Bgc = HLA-A28
- HLA antigens on RBCs are not destroyed by enzymes or DTT/AET treatment.
- However, Chloroquine or EDTA/glycine-HCL can be used to remove the HLA
antigen from RBCs. - Bg antibodies are usually considered insignificant, but although rare, Bg
antibodies have been reported to cause immediate and delayed HTRs. - HLA antibodies have not been implicated in HDFN.
- HLA antibodies play a significant role in transfusion-associates acute lung injury (TRALI).
A
HLA Antigens on RBCs
37
Q
- When an uncommon antibody is detected, understanding antigen and antibody characteristics and how they relate to frequency, ethinicity, enzymes, and chemical treatments is critical in the identification process.
- Antibodies to low-prevalence antigens are most often detected through
unexpected incompatible crossmatches or case of HDFN. - Antibodies to high-prevalence and othe uncommon antigens are easy to detect but difficult to work with since most blood banks do not have the antigen- negative reagent RBCs needed to exclude other alloantibodies, nor do they have typing reagents to phenotype the patient’s RBCs or havee an inventory of
rare units.
A
Applications to Routine Blood Banking
