The blood group antigen factsbook second edition

The Kell protein was reduced in one case of ITP5. The Kpa antigen in cis weakens the expression of Kell antigens cis-modifying effect 6, Kmod is an umbrella term used to describe various phenotypes with very weak expression of Kell antigens and increased expression of Kx antigen.

Antibodies produced by Kmod individuals are not necessarily mutually compatible. Antibodies to antigens in the Kell blood group system cause suppression of erythropoiesis in the fetus USA 88, — Lee, S. Williamson, L. Yu, L. Yazdanbakhsh, K. Vaughan, J. Expression of K can be acquired as a result of bacterial activity in vivo and in vitro. References 1 2 3 Lee, S. Weakened in rare genetic variants. Weak expression with concomitant Val1. Reference 1 Lee, S.

For the molecular basis associated with an absence of Ku K0 phenotype see Kell system pages. Kmod people make Ku-like antibodies that are not necessarily mutually compatible. Reference 1 Le Pennec, P. Jsa was shown to belong to the Kell system in Occurrence Caucasians Blacks 0.

Jsa is extremely sensitive to thiol reagents it is sensitive to 2 mM DTT most likely because it is located between two cysteine residues. Gordon, M. References 1 2 Lee, S. Stanworth, S. Named after the last letters of the antibody producer Karhula Kell blood group system Occurrence Less than 0.

Sakuma, K. No data although Mrs. Wallace, M. In , this antibody was named anti-K16 and the antigen K Chromium survival studies showed accelerated RBC destruction in one case2. Barrasso, C. Both McLeod and K0 phenotype blood will be compatible. Marsh, W. Found in one Italian family. The only example of the antibody was found in serum BUS by an incompatible crossmatch1.

References 1 2 Jongerius, J. References 1 2 Jones, J. Lewis antigens are not intrinsic to RBCs but are located on type 1 glycosphingolipids that are adsorbed onto blood cells from the plasma. See Section III. Patients with fucosidosis may have increased expression of Lewis antigens in their saliva and on their RBCs.

Glycoconjugates with Leb activity mediate attachment of Helicobacter pylori, a major causative agent of gastric ulcers, to gastric mucosal epithelium. See H [] chapter.

During pregnancy, expression of Lewis antigens on RBCs is often greatly reduced6. Lex and Ley are isomers of, respectively, Lea and Leb and often occur in sialylated forms. Sialyl Lex is a major neutrophil ligand for E-selectin8. Lea, Lex, their sialyl derivates, and also Leb and Ley, accumulate in tumor tissues.

Evidence indicates that adhesion of tumor cells to endothelial cells is mediated between the sialylated Lea and Lex antigens and E-selectin and represents an important factor in hematogenous metastasis of tumor cells. Anti-Lex and anti-Ley reagents are murine monoclonal antibodies made in response to immunization with various tumor cells.

Hauser, R. Hirschberg, C. Luhn, K. Fisher, T. Henry, S. Lowe, J. Walz, G. Reference 1 Henry, S. It is possible that a distinct Leab determinant with only Lewis blood group system weak affinity for anti-Lea is formed early in embryonic development. Anti-Leab is inhibited by saliva that contains Lea, and is weakly inhibited by saliva that contains Leb.

Reference 1 Schenkel-Brunner, H. Comments ALeb is often referred to as A1Leb because the transferase produced by the A1 gene is more efficient than the A2 transferase at adding sufficient quantities of N-Acetyl-D-galactosamine for detection by anti-ALeb. ALeb can also be adsorbed onto lymphocytes. Anti-ALeb is a single specificity that cannot be separated into anti-A1 and anti-Leb. Monoclonal anti-ALeb have been produced; some cross-react with the isomer ALey.

Comments BLeb can be adsorbed onto lymphocytes. Anti-BLeb is a single specificity; it cannot be separated into anti-B and anti-Leb.

Clears proinflammatory peptides3,4. Disease association Receptor for Plasmodium vivax, P. Unusual: Fyx haplotype expresses weak Fyb antigen not detected by all anti-Fyb. References 1 2 3 4 5 6 7 8 Pogo, A. Iwamoto, S. Darbonne, W. Neote, K. Pierce, S. Tournamille, C. Zimmerman, P. USA 96, — Rios, M. Duffy, who was a transfused hemophiliac. Comments Fya has been demonstrated on fetal RBCs as early as 6 weeks gestation.

Adult level of Fya expression attained approximately 12 weeks after birth. Fya antigen found on RBCs of baboon, but not chimpanzee, gorilla, gibbon, rhesus, cynomolgus, squirrel monkey, capuchin, douroncoli. References 1 2 3 4 5 Chaudhuri, A. Harris, T. Comments Poor immunogen. Fyb found on RBCs from chimpanzee, gorilla, gibbon, rhesus, cynomolgus, baboon; not found on RBCs from squirrel monkey, capuchin, dourocoli.

References 1 2 3 4 Chaudhuri, A. See system pages for molecular basis associated with an absence of Fy3. The third extracellular loop contains sequences necessary for binding of monoclonal anti-Fy3. Fy3 is expressed on RBCs from chimpanzees, gorilla, gibbon, rhesus, cynomolgus, baboon, dourocoli. The reactivity was enhanced with papain treated RBCs. Reference 1 Colledge, K. This antigen, although numbered by the ISBT, has only been defined by murine monoclonal antibodies.

Fy6 is expressed on RBCs from chimpanzee, gorilla, gibbon, squirrel monkey, dourocoli. Fy6 is not expressed on RBCs from rhesus, cynomolgus, baboon, capuchin.

References 1 2 Wasniowska, K. Pogo, A. References 1 2 3 4 5 6 7 8 9 10 Lucien, N. Sands, J. Irshaid, N. Lucien, N.

Sidoux-Walter, F. Mougey, R. Obarski, G. Name derived from the initials of the sixth child of the antibody maker, Mrs. Comments Jka has been demonstrated on fetal RBCs as early as 11 weeks gestation. Anti-Jka fades in vitro and in vivo. Often found in multi-specific sera. Reference 1 Yates, J. Anti-Jkb fade in vitro and in vivo. Med 8, — Comments Anti-Jk3 has been found in a non-transfused male.

People with In Jk do not make anti-Jk3 and the molecular basis of this phenotype is unknown. Diego was described in by Layrisse et al. N-terminus cytoplasmic domain residues 1— : Anchored to the membrane skeleton via ankyrin and protein 4. Disease association1 A severely hydropic baby, who lacked band 3 and protein 4.

Products of variant alleles of band 3 have been implicated in the pathogenesis of South East Asian ovalocytosis, congenital acanthocytosis, hereditary spherocytosis and distal renal tubular acidosis. None of the alleles encoding variants of the Diego blood group system are associated with disease. Band 3 has a role in the attachment of malarial parasites to the surface of RBCs and in adhesion of parasitized cells to the vascular epithelium. Altered form of band 3 present in South East Asian ovalocytes due to a deletion of amino acid residues — Band 3 Memphis has a Lys56 to Glu change.

References 1 2 3 4 Bruce, L. Schofield, A. Zelinski, T. Tanner, M. Dia with Lys 56 has been found in Amazonian Indians2. Examples of agglutinating anti-Dia and naturally occurring anti-Dia exist, but are rare.

References 1 2 Bruce, L. Baleotti, W, Jr. Reference 1 Bruce, L. It is frequently found in multispecific sera and is a common specificity in patients with AIHA. However, a strong anti-Wrb made by an untransfused, previously pregnant woman, with the Mi. V, Wr a-b- phenotype, gave results in the chemiluminescence assay that suggested the anti-Wrb was likely to cause accelerated destruction of transfused incompatible RBCs5. Fairly common specificity in patients with AIHA.

Wrb is not expressed on Ena. UK, GP. Hil, GP. SAT, GP. TK or GP. Dantu hybrid glycophorin molecules.

All have glutamic acid at residue of band 3 but lack the required amino acids from GPA6. References 1 2 3 4 5 6 Poole, J. Bruce, L. In the same study, anti-Wda was found in the serum of 1 of pregnant women. No other data are available.

Comments Anti-Wda is a common specificity in multi-specific sera. References 1 2 3 Bruce, L. Jarolim, P. Lewis, M. Redelberger, a donor and donor recruiter; assigned to Diego blood group system in Occurrence Found in three families. Comments Common specificity in multi-specific sera. References 1 2 Coghlan, G. Reference 1 Zelinski, T. Comments Anti-Wu is often found in multi-specific sera and may be naturally-occurring. Several members in one family of Dutch descent are likely to be homozygous for Wu.

References 1 2 Zelinski, T. Kaita, H. The band 3 amino acid substitution associated with expression of Bpa antigen is likely to be located within the RBC lipid bilayer; thus, the enzyme sensitivity of Bpa is somewhat surprising and may indicate the interaction with another enzyme sensitive component in the formation of the Bpa epitope.

Comment Anti-Moa may be naturally-occurring and is found in multi-specific sera. Comments Anti-Hga is found in multispecific sera; anti-Hga as a single specificity has not been reported.

Comments Anti-Vga is a relatively common antibody 11 examples of anti-Vga were found among donor sera and is found in multi-specific sera that frequently also contain anti-Wra. Anti-Swa and anti-Fra when present in the same serum show cross-reactivity and cannot be separated by absorption.

References 1 2 3 Zelinski, T. Comments Several examples of immune monospecific anti-BOW exist and it is often found in multi-specific sera.

The serological relationship to Wu2 cannot be explained by the molecular knowledge, although the critical residue Ala for Wu is relatively close to residue of band 3. References 1 2 McManus, K. The serological relationship to Wu2 cannot be explained by the molecular knowledge although the critical residue Ala for expression of Wu is relatively close to residue of band 3. The majority are naturally-occurring.

Reference 1 Poole J. Occurrence Only found in two English probands. Comments Anti-Tra was found as a separable specificity in 12 of 18 sera that contained anti-Wra. References 1 2 Jarolim, P. Anti-Fra and anti-Swa, when present in the same serum show cross-reactivity and cannot be separated by absorption2. See Swa DI14 for more details. AChE is in many tissues in various forms as a result of alternative splicing and post-translational modification. Function in RBC unknown.

Levels are reduced in myelodysplasias associated with chromosome 7 abnormalities and in some cases of SLE. References 1 2 Bartels, C. Rao, N. A second silent mutation in exon 5 does not correlate with the Yt polymorphism. Mazzi, G. The second example of anti-Ytb was made by a patient with PNH.

Reference 1 Bartels, C. Most abundant expression is in the most immature stages of the B cell, T cell and granulocyte lineages. Exon 1 to exon 3 are present in the pseudoautosomal region of the X and the Y chromosomes.

Exon 4 to exon 10 are only on the X chromosome. Family studies with anti-Xga helped to define the mechanism responsible for various sex-chromosome aneuploides. Xga and CD99 escape X-chromosome inactivation. XG gene transcripts were detected in thymus, bone marrow and fetal liver, and in several non erythroid tissues: heart, placenta, skeletal muscle, prostate, thyroid, spinal cord, trachea4. References 1 2 3 4 Latron, F.

Fouchet, C. And detected an antigen with a higher prevalence in females than in males; encoded by a locus on the X chromosome. Comments Some anti-Xga are naturally-occurring.

Rarely occurs with other alloantibodies. Xga is a poor immunogen. Xga has a phenotypic relationship with CD99, see system pages. Xga escapes X-chromosome inactivation. Xga is found on RBCs from gibbons but not on RBCs from chimpanzees, gorillas, orangutans, baboons, celebes black apes, various monkeys, mice, dogs and coelacanth. SC encodes a leader sequence of 29 amino acids. Disease association Not known.

The intracellular B References 1 2 3 4 5 Su, Y. Xu, H. Spring, F. Devine, P. Some examples are reactive in tests using serum but not plasma3. References 1 2 3 Wagner, F. Owen, I. Tregellas, W. Reference 1 Wagner, F. References 1 2 3 Peloquin, P. McCreary, J. Woodfield, D. Gene1—3 Chromosome Name Organization 12p Antigen mutation is numbered by counting Met as 1. Other monoclonal anti-Do agglutinate RBCs from greater and lesser apes.

Koch-Nolte, F. Grahnert, A. Smart, E. Scofield, T. Bailly, P. Poor immunogen; rarely found as a single specificity. Reference 1 Gubin, A. References 1 2 Banks, J. Vox Sang. Joined the Dombrock system in Reference 1 Reid, M. The original and second probands were later shown to be Hy—! The third proband had a JO allele and made anti-Joa. Transfusion 43, — Should have been named Calton, but the handwriting on the tube was misread Expression Tissues: Apical surface of proximal tubules, basolateral membranes subpopulation of collecting ducts in cortex, descending tubules in medulla, liver bile ducts, gall bladder, eye epithelium, cornea, lens, choroid plexus, hepatobilary epithelia, capillary endothelium1.

References 1 2 3 4 5 6 7 8 9 10 Preston, G. Smith, B. King, L. Preston, G. Chretien, S. Joshi, S. Nance, S. Agre, P. London , 3— Kozono, D. Should have been named Calton, but the handwriting on the tube was misread Occurrence All populations Kurtz, S. Covin, R. Simpson, W. Reference 1 Smith, B. Comments Poor immunogen, rarely found as a single specificity.

However, the phenotypic relationship between LW and the RhD antigen delayed recognition that LW was an independent blood group system until when it was named to honor Landsteiner and Wiener who made anti-LW in rabbits and guinea pigs after immunizing them with blood from Macacus rhesus. In , it became a three-antigen system. LW encodes a leader sequence of 30 amino acids. Possible marker for lymphocyte maturation or differentiation. May assist in stabilizing erythroblastic islands during erythropoiesis.

May be involved in removal of senescent RBCs3. LW antigens have been detected on RBCs of chimpanzee, gorilla, orangutan, baboon and various species of monkey but not on RBCs of rabbit, mouse, rat, sheep, goat, horse and cattle. References 1 2 3 4 5 6 7 8 9 Sistonen, P. Hermand, P. Zennadi, R. Blood Suppl. Bloy, C. Common in serum of patients with warm AIHA. Reference 1 Hermand, P. When LW antigens are suppressed, the anti-LWab may mimic an alloantibody and is a more common specificity than autoanti-LWa.

Reference 1 Storry, J. Anti-Ch was reported in and when anti-Rg was described in , there were obvious similarities between them. Ch and Rg appeared to be RBC antigens and were given blood group system status. C4A binds preferentially to protein and C4B to carbohydrate. C4d is a tryptic fragment of C4. The different antigens are usually identified in plasma by hemagglutination inhibition studies.

C4B binds more effectively to the RBC surface through sialic acid and thus is more effective at promoting hemolysis. A single amino acid substitution at position aspartic acid for histidine converts the functional activity of C4B to C4A2, whereas the substitution of cysteine for serine at position affects hemolytic activity and IgG binding.

Disease association Inherited low levels of C4 may be a predisposing factor for diseases such as insulin-dependent diabetes and autoimmune chronic active hepatitis. Comments Antigens of this system are stable in stored serum or plasma. Phenotypes and antibodies of this system are most accurately defined by hemagglutination inhibition tests. Sialidase-treated RBCs do not take up C4. Carroll, M. Virtually all anti-Ch contain anti-Ch1. References 1 2 Giles, C. Fisher, B. Giles, C. Detected on all C4B allotypes.

Comments Rare specificity, two examples reported. Antigen expression requires serine at residue , valine at and leucine at —3. Comments Rare specificity, two examples reported4. References 1 2 3 4 Giles, C. Comments All anti-Rg contain anti-Rg1 strongest component and anti-Rg2.

This transferase adds fucose to galactose on type 1 chains secretory tissues. The FUT1 product, a fucosyltransferase, attaches a fucose to galactose on type 2 carbohydrate chains attached to proteins or lipids. Disease association Increased expression with hematopoetic stress. Weakened expression in acute leukemia and carcinomatous tissue cells. Unusual: Para-Bombay. People having these h alleles in the homozygous state have Bombay or ParaBombay phenotypes.

Oh is associated with Tyr substituted by a stop codon. Several mutations give rise to the Para-Bombay phenotype9, Fernandez-Mateos, P. Kaneko, M. Wang, B. Koda, Y. Johnson, P. Kelly, R. Chee, K. Ogasawara, K. Hidalgo, A. Anti-IH is commonly found in the serum of pregnant group A1 women. Type-specific blood will be crossmatch compatible. Reference 1 Lowe, J. Amino acid sequence1 Note: the amino acid residues and listed here are the corrected data in GenBank accession number Z, which has not yet appeared in published form.

Involved in maintenance of normal cell membrane integrity. Disease association Absence of XK protein is associated with acanthocytosis and the McLeod syndrome, which manifests a compensated hemolytic anemia, elevated serum creatinine kinase and neuromuscular disorders including chorea, areflexia, skeletal muscle atrophy and cardiomyopathy3,4. See web site: www. See tables in Kell blood group system section.

Molecular bases of McLeod phenotype4,5 Unless otherwise stated found in one proband. References 1 2 3 4 5 6 7 Ho, M. Stanfield, G. Cell 5, — Danek, A. Russo, D. Hanaoka, N. Molecular basis associated with Kx antigen Not known. For molecular basis associated with a lack of Kx antigen, see table in system pages. XK is subject to X-chromosome inactivation and female carriers have a mixed population of normal and acanthocytic RBCs.

Contributes to the negatively charged glycocalyx. RBC receptors for influenza A and influenza B. The majority of RBC samples with Leach or Gerbich phenotypes have a weak expression of Kell blood group system antigens.

Gerbich antigens are weak on protein 4. GPC variants have been described that have amino acids encoded by duplicated exon 2 or duplicated exon 3. Reference 1 Colin, Y. The reciprocal gene to GYPC. Yus encodes two copies of amino acids encoded by exon 2.

The defining antibody was termed anti-Ge1,2,3 and later renamed to anti-Ge3. Occurrence Most populations Melanesians Alloanti-Ge3 can be made by individuals with either Gerbich or Leach phenotypes. Arndt, P. Transfusion 42 Suppl. Over fully updated entries on blood group antigens, formatted on single pages for easy use Inclusion of RHAG blood group system and over twenty new antigens Basic science paired with clinical applications to give context to information Full-color illustrations, gene maps and charts Both traditional and ISBT-sanctioned naming conventions included.

Most of the subsequent progress in immunogenetics was achieved by British investigators. The six consecutive editions of the unequaled Blood Groups in Man have long been considered as the bible of blood groupers.

It is quite unfortunate that this book has not been revisited since Although one cannot do without immunogenetics, which remains useful for the identification of new blood groups and genetic studies, the focus of interest has moved somewhat today. After several decades, the molecular basis of blood groups can be investigated by biochemists.

From to , the ABO, Hh, and Lewis blood groups served as models and their chemical basis came to be established. The red cell membrane glycophorins carrying the MN and Ss antigens and the glycolipids with P blood group specificities were also identified and characterized.

The chemical basis of the other groups, however, remained largely unknown. Popular Books. If you wish to place a tax exempt order please contact us. Add to cart. Sales tax will be calculated at check-out. Free Global Shipping.