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2330  Part XIII:  Transfusion Medicine                   Chapter 136: Erythrocyte Antigens and Antibodies            2331





                   TABLE 136–1.  Characteristics of International Society of Blood Transfusion Defined Blood Group Systems and Antigen
                   Collections (Continued)
                                                                                       Function of RBC
                                ISBT                   ISBT Gene                       Membrane
                   Conventional   Symbol   Chromosome   Name (ISGN If   Associated Antigens   Component
                   Name         (No.)     Location     Different)    [Null Phenotype]  (CD No.)        Disease Association
                   BLOOD GROUP SYSTEMS
                   ABO          ABO (001)  9q34.2      ABO           A, B, A, B, A1    Glycocalyx      Altered expression in
                                                                     [group O]                         some hematologic
                                                                                                       disorders
                   MNS          MNS (002)  4q31.21     MNS (GYPA,    M, N, S, s, U, and 43   Binds microbe    Decreased Plasmodium
                                                       GYPB)         more [En(a–); U–;   glycocalyx,    falciparum invasion, may
                                                                     M M ]             complement regu-  be receptor for Escherichia
                                                                      k
                                                                        k
                                                                                       lation, chaperone   coli
                                                                                       for band 3 (CD235)
                                                                        k
                   P1PK         P1PK (003)  22q13.2    P1 (A4GALT)   P1, P , NOR       Glycocalyx
                   Rh           RH (004)  1p36.11      RHD, RHCE (RH)  D, C, E, c, e, and 49   Possibly transports  Hemolytic anemia, hered-
                                                                     more [Rh ]        CO  or NH  (CD240) itary stomatocytosis,
                                                                            null         2    3
                                                                                                       hematologic malignancies
                   Lutheran     LU (005)  19q13.32     LU            Lu , Lu , Lu3, and   Binds laminin   Increased expressions
                                                                          b
                                                                       a
                                                                     18 more [recessive   (CD239)      possibly involved in
                                                                     Lu(a–b–)]                         vasoocclusion in sickle cell
                                                                                                       disease
                   Kell         KEL (006)  7q34        KEL           K, k, Kp , Kp , Js , Js ,   Cleaves big endo-
                                                                          a
                                                                                   b
                                                                                a
                                                                             b
                                                                     and 29 more [K  or   thelin-3 to ET-3, a
                                                                     K ]        0      potent vasocon-
                                                                      null
                                                                                       strictor (CD238)
                                                                      a
                                                                                                 b
                                                                         b
                   Lewis        LE (007)  19p13.3      LE (FUT3)     Le , Le , and 4 more   Glycocalyx, Le  is   Increased expression in
                                                                     [Le(a–b–)]        receptor for Helico-  fucosidosis, Lewis anti-
                                                                                       bacter pylori   bodies may be important
                                                                                                       in graft rejection
                   Duffy        FY (008)  1q23.2       FY (DARC)     Fy , Fy , Fy3, Fy5, Fy6   Chemokine, Plas-  Resistance to P. vivax
                                                                         b
                                                                      a
                                                                     [Fy(a–b–)]        modium vivax    invasion
                                                                                       receptor (CD234)
                   Kidd         JK (009)  18q12.3      JK(HUT11,     Jk , Jk , Jk3 [Jk(a–b–)]  Urea transporter  Impaired urea transport,
                                                                      a
                                                                         b
                                                       SLC14AI)                                        urine-concentrating
                                                                                                       defect
                   Diego        DI (010)  17q21.31     DI (SLC4A1; AE1)  Di , Di , Wr , Wr , and   Anion exchanger   Southeast Asian ovalocy-
                                                                             a
                                                                         b
                                                                      a
                                                                                b
                                                                     18 more           (CD233), Band   tosis, hereditary sphero-
                                                                                       3 cytoskeletal   cytosis, renal tubular
                                                                                       protein         acidosis
                   Yt           YT(011)   7q22.1       Yt (ACHE)     Yt , Yt b         Acetylcholin-   Absent from PNH III RBCs
                                                                      a
                                                                                       esterase
                   Xg           XG (012)  Xp22.33      XG (XG, MIC2)  Xg , CD99        Adhesion mole-
                                                                       a
                                                                                       cules (CD99)
                   Scianna      SC (013)  1p34.2       SC (ERMAP)    Sc1, Sc2, Sc3, and 4   Possible adhesion
                                                                     more [SC: –1, –2, –3]
                   Dombrock     DO (014)  12p12.3      DO (ART4)     Do , Do , Gy , Hy, Jo   a  Enzymatic (CD297)  Absent from PNH III RBCs
                                                                              a
                                                                          b
                                                                       a
                                                                     and 3 more [Gy(a–)]
                                                                          b
                                                                       a
                   Colton       CO (015)  7p14.3       CO (AQP1)     Co , Co , Co3, Co4   Water transport  Monosomy 7, inability to
                                                                     [Co(a–b–)]                        maximally concentrate
                                                                                                       urine, congenital dysery-
                                                                                                       thropoietic anemia
                   Landsteiner-   LW (016)  19p13.2    LW (ICAM4)    LW , LW , LW      Binds CD11/CD18,   Depressed in pregnancy
                                                                       a
                                                                          ab
                                                                              b
                   Wiener                                            [LW(a–b–)]        ligand for integrins  and some malignant
                                                                                       (CD242)         diseases
                   Chido/Rogers  CH/RG    6p21.32      C4A,C4B       CH1, RGl, and 7 more  Complement   Certain phenotypes have
                                (017)                                                  components      increased susceptibility to
                                                                                                       certain autoimmune con-
                                                                                                       ditions and infections
                                                                                                                   (continued )
          Kaushansky_chapter 136_p2327-2352.indd   2331                                                                 9/21/15   4:30 PM
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