UNCOMMON BLOOD GROUPS P3

Question 1

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

Answer 1

The CD59 (035) System

Question 2

(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.

Answer 2

chromosome 11 ;
CD59 ; membrane inhibitor lysis (MIRL) ;
PNH ;
Congenital CD59-deficient

Question 3

(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.

Answer 3

Anti-CD59.1

Question 4

• 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.

Answer 4

The Augustine (036) System

Question 5

(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.

Answer 5

Ata ;
AUG1 and AUG2 ;
chromosome 6 ; equilibrative nucleoside transporter 2 (ENT2) ;

Question 6

(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.

Answer 6

AUG3

Question 7

• 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.

Answer 7

ISBT Blood Group Collections

Question 8

• formally referred to as Cost-Sterling, was established in 1988.
• It was named after the two patients (Copeland and Sterling) who made anti-Csa.

Answer 8

Cost Collection 205

Question 9

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.

Answer 9

complement receptor 1 (CR1). ;
Csa and Csb. ;
Csa ;
Csb ;

Question 10

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.

Answer 10

Cost Collection

Question 11

• 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.

Answer 11

Ii Collection 207

Question 12

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.

Answer 12

Ii Collection 207

Question 13

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.

Answer 13

Ii Collection 207

Question 14

was established as a collection in 1990 with three antigens.

Answer 14

Er Collection 208

Question 15

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.

Answer 15

Era and Er3 ;
Erb

Question 16

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.

Answer 16

Anti-Er3

Question 17

• 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.

Answer 17

Globoside Collection 209

Question 18

• 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.

Answer 18

Collection 210 (Unnamed)

Question 19

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.

Answer 19

Lec ;
Led

Question 20

• ______ 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.

Answer 20

Anti-Lec ;
Anti- Led

Question 21

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.

Answer 21

Hu,M1, Tm, Can, Sext, and Sj. ;
GPA ;
Hunter

Question 22

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.

Answer 22

M antigen ;

Question 23

• 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.

Answer 23

Sheerin

Question 24

• 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.

Answer 24

can

Question 25

• 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.

Answer 25

Sext

Question 26

• 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

Answer 26

Sj

Question 27

(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.

Answer 27

room temperature

Question 28

• 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.

Answer 28

ISBT 700 Series

Question 29

• 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.

Answer 29

ISBT 901 Series

Question 30

• 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.

Answer 30

Emm Antigen

Question 31

• 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.

Answer 31

AnWj Antigen

Question 32

• 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.

Answer 32

Sda Antigen

Question 33

• 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.

Answer 33

PEL Antigen

Question 34

• 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.

Answer 34

ABTI Antigen

Question 35

• 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.

Answer 35

MAM Antigen

Question 36

• 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).

Answer 36

HLA Antigens on RBCs

Question 37

• 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.

Answer 37

Applications to Routine Blood Banking