Week 10 Blood Groups (PDF)

10 OTHER Minor BLOOD GROUPS Diego Blood Group System (010) Dia antigen has served as a useful tool in anthropologic studies of Mongolian ancestry Dia and Dib antigens are located on the anion exchange molecule (AE-1), which is an integral Dia transport protein involved in the anion exchange of Wda b...

10 OTHER Minor BLOOD GROUPS Diego Blood Group System (010) Dia antigen has served as a useful tool in anthropologic studies of Mongolian ancestry Dia and Dib antigens are located on the anion exchange molecule (AE-1), which is an integral Dia transport protein involved in the anion exchange of Wda bicarbonate for chloride in the red blood cell Dib membrane Rba Wra Defects of AE-1: WARR hereditary spherocytosis Wrb congenital acanthocytosis Southeast Asian ovalocytosis Both anti-Dia and anti-Dib are characterized as red cel|-stimulated, |gG antibodies that do not bind the complement and are reactive in the indirect antiglobulin testing, A Antithetical Low Incidence Antigens Antigens Wright Antigens Wright antigens were first classified as an independent blood group system and later on as a collection Two types of anti-Wra have been observed: a naturally occurring IgM immune-stimulated IgG Cartwright Blood Group System (011) Yt Antigens located on the erythrocyte acetylcholinesterase, which is an enzyme involved in neurotransmission Yta high-incidence antigen present in 99.8% of the general population Ytb low-incidence antigen present in 8% of the general population Yt Antibodies: anti-Yta & anti-Ytb reactive in indirect antiglobulin test considered to be clinically important because they are predominantly red cell stimulated Xg Blood Group System (012) Xga Antigen Only the Xga antigen has been identified with no known antithetical partner Gene that codes for the Xg allele is located on the short arm of the X chromosome Xga antigen is carried by a protein with cell adhesion properties that have been demonstrated to have homology with the CD99 molecule Difference in the frequency of the Xg' antigen is noted between the sexes: Female: 89% Male: 66% Anti Xga Antibodies predominantly IgG reactive in the indirect antiglobulin testing sensitive to enzymes but not dithiothreitol treatment Most antibodies are the result of red cell stimulation but have not been associated with causing HTR and HDFN Scianna Blood Group System (013) Sc Antigens composed of Sc1, Sc2and Sc3 antigens anti-Sc1 and anti-Sc2 IgG, red cell-stimulated and react in indirect antiglobulin testing both have been implicated in transfusion reactions Anti-Sc3 characterized as IgG, red cell-stimulated, and react in the indirect antiglobulin phase of testing has been linked to causing mild transfusion reactions Sc -1,-2,-3 rare null phenotype has been observed in the Marshall Islands and New Guinea Dombrock Blood Group System (014) Antigens Doa, Dob: carried on Mono-ADP-Ribosy|transferase 4 Gregory (Gya), Holley (Hy) and Joseph (Joa) Antibodies 1. Anti-Doa and anti-Dob described as gG, red cell-stimulated antibodies react primarily in the indirect antiglobulin test with polyethylene glycol (PEG) or enzyme enhancement neither associated with clinical HDFN but both have been reported to cause acute to delayed transfusion reactions 2. Anti-Gya, anti-Hy, anti-Joa and anti-Dob characterized as gG, red cell-stimulated, and indirect antiglobulin test reactive no cases of clinical HDFN have been described all have been associated with causing moderate transfusion reactions Colton Blood Group System (015) Antigens Coa, Cob and Co3 (formerly Coab) The Colton antigens are carried on an integral membrane protein, aquaporin 1 (AQP1), which accounts for 80% of water reabsorption in the kidneys. CO antigens have been located on the transport protein known as channelforming integral protein (CHIP), which forms the primary erythrocyte water channel and is responsible for water permeability CHIP and the CO antigens are expressed in the tissues of the proximal and descending tubules and the collecting ducts of the kidney and are believed to account for 80% of the reabsorption of water Antibodies 1. Anti-Coa and anti-Cob IgG, red cell-stimulated and reactive in indirect antiglobulin testing associated with acute to delayed transfusion reactions 2. Anti-Co3 characterized as IgG red cell-stimulated complement binding reacting in the indirect antiglobulin phase testing antibody has been associated with causing severe HDFN Landsteiner-Wiener Blood Group System (016) Antigens Antigen carried by intracellular adhesion molecule 4 (ICAM4) LW has a phenotypic relationship with the D antigen Rhnull RBCs type LW(a-b-) Antibodies Anti-LW reacts strongly with D+ RBCs and can look like anti-D originally identified as anti-Rh; different from anti-D Anti-LW differs from anti-D, where: Anti-LW agglutinates Rh (+) and Rh (-) cells except Rhnull Anti-LW: no reaction with DIT treated RBCs; anti-D reacts with DIT treated RBCs Chido/Rodgers Blood Group System (017) Antigens Includes nine antigens which are subdivided into six Chido antigens, two Rodgers antigens, and the WH antigen It was postulated that the CH/RG antigens were associated with the human leukocyte antigen (HLA) system Alleles for Rg and Ch have been located on two closely linked genes known as C4A and C4B on chromosome 6 Antigen products have been demonstrated as on the C4d fragments of the C4A (Rodgers) and C4B (Chido) glycoproteins of the C4 complement component Antibodies Formerly, the anti-Ch/Rg antibodies were collectively grouped as high-titer, low-avidity (HTLA), along with other antibodies sharing common serologic properties. Anti-Ch/Rg antibodies are usually stimulated in multi -transfused individuals lacking one or more of the corresponding antigens; these antibodies are IgG and weakly reactive in the indirect antiglobulin phase of testing Gerbich Blood Group System (020) Antigens GE antigens are inherited on chromosome 2 and are expressed on glycophorins C (GPC) and/or D (GPD) High Incidence: Ge2, Ge3 and Ge4 Low Incidence: Wb, Lsa, Ana and Dha Gerbich phenotype Ge:-2, -3, 4 Papua New Guinea, Europeans, Africans, Native Americans, Japanese, and Polynesians Yus phenotype Ge:-2, 3, 4 Mexicans, Israelis Ge:-2, -3, -4 Gerbich null within Papua New Guinea Leach phenotype Individuals of Leach phenotype (Ge:-2, -3, -4) present with change in electrolyte morphology in the form of elliptocytosis Antibodies 1. Anti-Ge2 and anti-Ge3 naturally occurring IM immunoglobulins and as autoantibodies implicated in causing acute to delayed transfusion reactions but not in cases of clinical HDFN. 2. anti-Wb, anti-Lsa, anti-Ana and anti-Dha described as predominantly IgG with an IgM component and unable to bind complement Cromer Blood Group System (021) Antigens high-incidence antigens low-incidence antigens Cra, Tca, Dra, Esa, IFC, UMC, and WESb Tcb, Tcc, and WESa Antigens are carried by decay accelerating factor (DAF), which is involved with the regulation of complement activation by accelerating the decay of the C3 and C5 convertases Antibodies 1. Anti-CROM described as predominantly IgG1 red cell-stimulated, and reactive in indirect antiglobulin testing neutralization may be accomplished with concentrated serum, plasma and urine rarely observed, with the majority of examples presenting in Black individuals Knops Blood Group System (022) Antigens Kna (Knops), Knb, McCa (McCoy), SIa, and Yka (York) Alleles for the KN blood group have been located on chromosome 1, with the antigens residing on complement receptor one (CR1) Antibodies characterized as gG, red cell-stimulated, and reacting weakly and variably at the indirect antiglobulin phase viewed to be of little clinical importance because none has been associated with causing HDFN or HTR Indian Blood Group System (023) Antigens Composed of two antithetical antigens, Ina and Inb which are of relatively low and high incidence, respectively IN antigens are carried on the hematopoietic isoform of the CD44 marker, which is known for its immune adhesion properties Antibodies IN antibodies are characterized as IgG, red cell-stimulated, and reactive in indirect antiglobulin testing OK Blood Group System (024) The OK antigens are carried on CD147, a member of the immunoglobulin superfamily that mainly functions as receptors and adhesion molecules Raph Blood Group System (025) MER2 is the only antigen MER2 is located on CD151, a tetraspanin, which appears to be essential for the assembly of basement membranes in the kidney and skin John Milton Hagen Blood Group System (026) JMH protein is glycophosphatidylinositol (GPI)-linked glycoprotein CD108 Gill Blood Group System (029) GIL is the only antigen This antigen is found on the glycerol transporter aquaporin 3 (AQP3), a member of the major intrinsic of water channels protein family Rh-associated glycoprotein (030) RhAg does not have Rh blood group antigens; its presence in a complex with the Rh proteins is essential for Rh antigen expression Two antigens have been definitively assigned to the RAG system: Duclos (RHAG1), previously 901013 in the high prevalence series OIa (RHAG2), previously 700043 in the low prevalence series Human Leukocyte Antigens (HLA) detectable on RBCs Antigens Bga HLA-B7 Bgb HLA-B17 Bgc HLA-A28 Antibodies characterized as IgG acting weakly and variably in indirect antiglobulin test not implicated in HDFN or HTR removed by chloroquine and glycine-HCI/EDTA MISCELLANEOUS ANTIGENS Vel Anti-Vel is most often IgG but can be IgM, and has caused severe immediate HTRs and HDFN When serum is tested, anti-Vel characteristically causes in vitro hemolysis Ata Anti-Ata has been found only in Blacks the antibody is usually IgG and has caused severe HTRs Jra is Anti-Jra is found more commonly in Japanese, but clinical significance is not well established, since it is a rare antibody it has caused HTRs and a fatal case of HDFN Sda Anti-Sda has characteristic shiny and refractile agglutinates under the microscope and is inhibited with urine from Sd (a+) individuals High-Incidence Antigens Unrelated to Principal Blood Group Systems Defined as antigens occurring in 99.9% of the population Examples of high-frequency antigens that are apparently unrelated to principal blood group systems include: Augustine (Ata) Cromer (Cra) Ena Gerbich (Ge) Langereis (Lan) Oka Gregory (Gya) Holley (Hy) Jacobs (Jra) Joseph (Joa) Vel Phenotypes that are predominantly found in Blacks At (a-) Cr (a-) Jo (a-) LOW-Incidence Antigens Unrelated to Principal Blood Group Systems Defined as antigens with an incidence of less than 1% Wright Swann (Swa) By Mta Tra High-Titer, Low-Avidity (HTLA) Antibodies Antibodies to high frequency antigens that are generally clinically benign (no HTRs nor HDFN) Antibodies exhibit reactivity at high dilutions of serum, but the strength of agglutination is weak at any dilution Antibodies formed classified as HTLAs owing to similar serologic reactivity: Anti-Cha (Chido) Anti-Rga (Rodgers) Anti-Kn(Knops-Helgeson) Anti-JMH (John Milton Hagen) Anti-Yka (York) Anti-Csa (Cost-Stirling) Anti-McCa (McCoy) Other Blood Group Antigens: Granulocyte antigens Human Neutrophil Antigen (HNA) and Human Granulocyte Antigens (HGA-3a to 3e) Clinical significance: Neonatal alloimmune neutropenia Autoimmune neutropenia Transfusion-related acute lung injury (TRALI) Platelet antigens Human Platelet Antigen (HPA): HPA-1 and HPA-4 = GpllIa HPA-2 = GpIb HPA-3 = GpIIb HPA-5 = Gpla Clinical significance: Neonatal alloimmune thrombocytopenia Post-transfusion purpura ANTIBODY CHARACTERISTICS Clinically significant Usually clinically insignificant Naturally occurring Warm antibodies Cold antibodies Usually only react in AHG Can react in any phase of testing Detection enhanced by enzyme treatment of test cells ANTIBODY CHARACTERISTICS Not detected with enzyme treatment of test cells Enhanced by acidification Show dosage Bind complement Cause in vitro hemolysis Labile in vivo and in vitro Common cause of anamnestic response (delayed HTR) ANTIBODY CHARACTERISTICS Associated with paroxysmal cold hemoglobinuria Associated with CAD and PAP Associated with infectious mononucleosis