OTHER BLOOD GROUP SYSTEM

• Kell (006) and Kx (019) Systems


  The first blood group system discovered after the introduction of antiglobulin testing
• Kell blood group antigens

  • Found only on RBCs
  • The Kell glycoprotein is covalently linked with another protein, called Xk
• K0


  Null phenotype
• Anti-K


  • Outside of the ABO and Rh antibodies, it is the most common antibody seen in the blood bank
  • Usually IgG and reactive in the antiglobulin phase
  • Naturally occurring IgM examples are rare, but have been associated E. coli O125:B15 infection
  • Has been implicated in severe HTRs and severe HDFN
• McLeod Phenotype


  • Weakened expression of the Kell antigens, designated by a superscript w for "weak"
  • Common to male
  • Associated with acanthocytes
  • Associated with Chronic Granulomatous Disease (CGD)
• Duffy (008) System


  • Destroyed by enzymes (ficin, papain, bromelin, and chymotrypsin, and ZZAP)
  • Anti-Fya and anti-Fyb have been associated with acute and delayed HTRs
  • Fy(a–b–) RBCs resist infection in vitro by the monkey malaria organism P. knowlesi, and P. vivax
• The amino acid at position 42 on the Duffy glycoprotein defines the Fya and Fyb polymorphism: Fya has glycine, and Fyb has aspartic acid
• The Duffy glycoprotein is a member of the superfamily of chemokine receptors and is known as the atypical chemokine receptor 1 (ACKR1, previously known as DARC)
• Kidd (009) System


  • A simple and straightforward system consisting of only three antigens (Jka , Jkb, Jk3)
  • The titer of anti-Jka or anti-Jkb quickly declines in vivo
  • A strong antibody identified following a transfusion reaction may be undetectable in a few weeks or months
  • Anti-Jk3 has been associated with severe immediate and delayed HTRs and with mild HDFN
• Lutheran (005) System


  Affected by enzymes: resistant to the enzymes ficin, papain and glycine-acid EDTA treatment, destroyed by treatment with the enzymes trypsin and α-chymotrypsin
• Diego (010) System


  • Antigens: Dia /Dib , Wra /Wrb, and Wu/DISK
  • Dia is rare in most populations but is polymorphic in people of Mongoloid ancestry
  • Diego system antibodies are sometimes IgM, but are usually IgG, reactive in the antiglobulin phase of testing
  • Both anti-Dia and anti-Dib have caused hemolytic transfusion reactions (HTRs) and HDFN
• Yt (011) System


  • Yta is the high-prevalence antigen in all populations
  • Ytb is the low-prevalence antigen
  • Not found in Japanese
  • The Yt antigens represent an amino acid substitution on the glycosylphosphatidylinositol (GPI) linked RBC glycoprotein acetylcholinesterase (AChE)
• Xg (012) System


  • Antigen has a higher prevalence in females than in males
  • Family studies were used to confirm the antigen Xga expression was controlled by an X-linked gene
  • There are two antigens in the Xg system: Xga and CD99
• Scianna (013) System


  • Currently consists of seven antigens
  • High prevalence antigen: Sm (Sc1)
  • Low prevalence antigen: Bua (Sc2)
  • SC gene is located on chromosome 1 at position 1p34.2
  • The product of the gene is a protein called erythroid membrane associated protein (ERMAP), which is an RBC adhesion protein
  • The very rare Sc:–1,–2,–3 phenotype is the Scianna null type
• Dombrock (014) System


  • The Dombrock antigens are carried on a mono-ADP-ribosyltransferase 4 (ART4) attached to the RBC membrane by a GPI anchor
  • The Dombrock antibodies are usually IgG
  • Poor immunogens: Doa and Dob
  • Highly immunogenic: Gya
• Colton (015) System


  • Consists of four antigens (Coa, Cob, Co3, Co4)
  • Coa is high prevalence, Cob is low prevalence
  • Co3 is present on all RBCs except those of the very rare Co(a–b–)phenotype
  • Co4 is a high-prevalence antigen, has been identified on RBCs from two individuals with the Co(a–b–) phenotype
  • The Colton antigens are carried on an integral membrane protein, aquaporin 1 (AQP1), which accounts for 80% of water reabsorption in the kidneys
• Landsteiner—Weiner (016) System


  • The structure that carries the LW antigens is a glycoprotein known as intracellular adhesion molecule 4 (ICAM-4), a member of the immunoglobulin superfamily
  • LW antigens may be depressed during pregnancy and in some diseases, such as lymphoma and leukemia
  • Autoanti-LW is also common in serum from patients with warm autoimmune hemolytic anemia
• Chido—Rodgers (017) System


  • Ch and Rg antigens are not intrinsic to the RBC membrane. Rather, they are on the fourth component of complement (C4), and are adsorbed onto RBCs from plasma
  • The C4 glycoprotein has two isoforms: C4B carries the Ch antigens, and C4A expresses Rg antigens
  • Antigens: Ch1 to Ch6, Rg1 to Rg2
  • Antibodies: Neutralized by the plasma
• Gerbich (020) System


  • The antigens are carried on sialoglycoprotein structures GPC and GPD
  • There are three Gerbich-negative phenotypes in which the RBCs lack one or more of the high-prevalence antigens: Ge:–2,3,4 (Yus type), Ge:–2,–3,4 (Gerbich type), Ge:–2,–3,–4 (Leach type)
  • The Leach type is the Gerbich null phenotype
• Cromer (021) System


  • The antigens of the Cromer system are carried on decay accelerating factor (DAF, CD55), a complement-regulatory protein
  • DAF is strongly expressed on placental tissue and will adsorb Cromer antibodies
• Knops (022) System


  The system was established when the
• C4B carries the Ch antigens, and C4A expresses Rg antigens
• Antigens


  • Ch1 to Ch6
  • Rg1 to Rg2
• Antibodies are neutralized by the plasma
• Gerbich (020) System


  The antigens are carried on sialoglycoprotein structures GPC and GPD
• Gerbich (020) System


  • The glycoproteins help to maintain the RBC membrane integrity through interaction with protein band 4.1 and p55
  • They are rich in sialic acid, they contribute to the net negative charge of the RBC membrane (as do GPA and GPB of the MNS system)
• Gerbich-negative phenotypes


  • Ge:–2,3,4 Yus type
  • Ge:–2,–3,4 Gerbich type
  • Ge:–2,–3,–4 Leach type
• The Leach type is the Gerbich null phenotype
• Cromer (021) System


  The antigens are carried on decay accelerating factor (DAF, CD55), a complement-regulatory protein
• DAF is strongly expressed on placental tissue and will adsorb Cromer antibodies
• Knops (022) System


  • The antigens are located on complement receptor 1 (CR1)
  • CR1 binds the complement component fragments C3b and C4b and processes immune complexes for transportation to the liver and spleen and subsequent clearance from the circulation
• The "Helgeson phenotype" represents the serologic null phenotype for the Knops blood group
• Indian (023) System


  The antigens are located on CD44, an adhesion molecule
• Ok (024) System


  • The OK antigens are carried on CD147, or basigin, a member of the immunoglobulin superfamily that mainly functions as receptors and adhesion molecules
  • Basigin is a receptor essential for invasion by P. falciparum
• Raph (025) System


  • The only antigen in the Raph system is MER2, whose name is derived from monoclonal, and Eleanor Roosevelt, the laboratory where the antibody was produced
  • MER2 is encoded by the gene CD151
• John Milton Hagen (026) System


  • The antibody was named anti-JMH for the first antibody maker, John Milton Hagen
  • Anti-JMH is usually IgG (predominantly IgG4 in acquired JMH-negative people)
• Gill (029) System


  The antigen is found on the glycerol transporter aquaporin 3 (AQP3), a member of the major intrinsic protein family of water and glycerol channels
• Rh-Associated Glycoprotein (030) System


  • The Rh-associated glycoprotein (RhAG) does not have Rh blood group antigens; however, its presence in a complex with the Rh proteins is essential for Rh antigen expression
  • Four antigens have been assigned to the RHAG system: Duclos (high prevalence), Ola (very rare, low prevalence), the high prevalence DSLK (for Duclos-like), and RHAG4
• FORS (031) System


  The Forssman glycolipid can serve as a receptor for pathogens such as Escherichia coli, making one believe that human cells that express the FORS1 may have an increased susceptibility to E. coli infection
• JR (032) System


  • Jr(a) is a high-prevalence antigen in most populations; the Jr(a–) phenotype is found more commonly in Japanese
  • Associated with ABCG2, a member of the adenosine triphosphate (ATP) binding cassette transporters broadly distributed throughout the body
  • It is involved in multidrug resistance in tumor cells, presenting a problem in chemotherapy