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Williams Hematology, 8e > Part XIII. Transfusion Medicine > Chapter 137.

Erythrocyte Antigens and Antibodies > Definitions and History > INTERNATIONAL SOCIETY OF BLOOD TRANSFUSION DEFINED BLOOD GROUP SYSTEMS AND ANTIGEN COLLECTIONS WITH CHROMOSOME AND GENE Conventional Name ISBT Symbol (No.) Blood Group Systems ABO ABO (001) MNS MNS (002) Chromosome Location ISBT Gene Name (ISGN If Different) ABO Associated Antigens [Null Phenotype] Function of RBC Membrane Component (CD No.) Glycocalyx Disease Association

9q34.2

A, B, A, B, A1 [group O] M, N, S, s, U, He, Mia, Vw, Mc, Mur, Mg, Vr, Mta Sta, Ria, Cla, and 29 more [En(a); U; Mk Mk] P1 D, C, E, e, e, f, Cw, Cx, V, G, hrs, VS, Dw, Rh29, Goa, hrB, Rh32, Rh33, and 32 more [Rhnull] Lua, Lub, Lu3, Lu4, Lu5, Lu6, Lu7, Lu8, Lu9, Lu11, Lu12, Lu13, and 7 more [recessive Lu(ab)] K, k, Kpa, Kpb, Ku, Jsa, Jsb, and 23 more [K0 or Knull] Lea, Leb, Leab, Lebh, ALeb, BLeb[Le(ab)]

4q31.21

MNS (GYPA, GYPB)

Binds microbe glycocalyx, complement regulation, chaperone for band 3 (CD235)

Altered expression in some hematologic disorders Decreased Plasmodium falciparum invasion, may be receptor forEscherichia coli

P Rh

PI (003) RH (004)

22q11.2-qter 1p36.11

P1 RHD, RHCE (RH)

Glycocalyx Possibly transports CO2 or NH3 (CD240)

Hemolytic anemia, hereditary stomatocytosis, hematologic malignancies Increased expressions possibly involved in vasoocclusion in sickle cell disease

Lutheran

LU (005)

19q13.2

LU

Binds laminin (CD239)

Kell

KEL (006)

7q34

KEL

Lewis

LE (007)

19p13.3

LE (FUT3)

Cleaves big endothelin-3 to ET-3, a potentvasoconstrictor(CD23 8) Glycocalyx, Leb is receptor Increased expression in forHelicobacter pylori fucosidosis, Lewis antibodies may be important in graft rejection
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International Society of Blood Transfusion Defined Blood Group Systems and Antigen Collections with Chromosome and Gene

Conventional Name Duffy

ISBT Symbol (No.) FY (008)

Chromosome Location 1q23.2

ISBT Gene Name (ISGN If Different) FY (DARC)

Associated Antigens [Null Phenotype] Fya, Fyb, Fy3, Fy4, Fy5, Fy6 [Fy(ab)] \ Jka, Jkb, Jk3 [Jk(ab)] Dia, Dib, Wra, Wrb, Wda, Rba, and 14 more Yta, Ytb Xga, CD99 Sc1, Sc2, Sc3, Rd [Sc: 1, 2, 3] Doa, Dob, Gy, Hy, Joa[Gy(a)] Coa, Cob, Co3 [Co(ab )]

Function of RBC Membrane Component (CD No.) Chemokine,Plasmodium vivaxreceptor (CD234)

Disease Association

Resistance to P. vivax invasion

Kidd

JK (009)

18q12.3

JK(HUT11, SLC4AI) DI (SLC4A1; AE1)

Urea transporter

Diego

DI (010)

17q21.31

Anion exchanger (CD233), Band 3 cytoskeletal protein

Yt Xg Scianna Dombrock

YT(O11) XG (012) SC (013) DO (014)

7q22 Xp22.33 1p34.2 12p12.3

Yt (ACHE) XG (XG, MIC2) SC (ERMAP) DO (ART4)

Acetylcholinesterase Adhesion molecules (CD99) Possible adhesion Enzymatic (CD297)

Impaired urea transport, urine concentrating defect Southeast Asian ovalocytosis, hereditary spherocytosis, renal tubular acidosis Absent from PNH III RBCs

Absent from PNH III RBCs Monosomy 7, inability to maximally concentrate urine, congenital dyserythropoietic anemia Depressed in pregnancy and some malignant diseases Certain phenotypes have increased susceptibility to certain autoimmune conditions and infections Decreased in some tumor cells, increased in hematopoietic stress
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Colton

CO (015)

7p14

CO (AQP1)

Water transport

LandsteinerWiener Chido/Rogers

LW (016)

19p13.2

LW (IC AM)

LWa, LWab, LWb [LW(ab)] CH1, CH2, Rgl, and Rg6 more

Binds CD11/CD18, ligand for integrins (CD242) Complement components

CH/RG (017)

6p21.32

C4A,C4B

Hh (018)

19q13.33

H (FUT1)

H [Bombay, Oh]

Glycocalyx (CD 173)

International Society of Blood Transfusion Defined Blood Group Systems and Antigen Collections with Chromosome and Gene

Conventional Name Kx

ISBT Symbol (No.) XK (019)

Chromosome Location Xp21.1

ISBT Gene Name (ISGN If Different) XK

Associated Antigens [Null Phenotype] Kx [McLeod]

Function of RBC Membrane Component (CD No.) Possible neurotransmitter, function in RBCs not known

Disease Association

Gerbich

GE (020)

2q14.3

GE (GYPC)

Ge2, Ge3, Ge4, Wb, Lsa, Ana, Dha[Leach phenotype] Cra, Tca, Tcb, Tcc, Dra, Esa, IFC, WESa, WESb, UMC, GUTI, SERF, CROV, CRAM [Inab phenotype] Kna, Knb, McCa, SIa, Yka, McCb, Vil, S13, KCAM [Helgeson phenotype] Ina, Inb, INFI, INJA

Membrane attachment; interacts with 4.1R and p55 (CD236) Complement regulation, binds C3b, disassembles C3/C5 convertase (CD55)

Cromer

CROM (021)

1q32.2

CROM (DAF)

Acanthocytosis, muscular dystrophy, hemolytic anemia; McLeod syndrome sometimes associated with CGD, peripheral neuropathy, cardiomyopathy seizures, a late-onset dementia, and behavioral changes Hereditary elliptocytosis, hemolytic anemia, decreased 4.1R and p55 Absent from PNH III RBCs, Dra is the receptor for uropathogenic E. coli

Knops

KN (022)

1q32.2

KN (CR1)

Complement regulation, binds C3b and C4b, mediates phagocytosis (CD35) Binds hyaluronic acid, mediates adhesion of leukocytes (CD44) Possible adhesion (CD147) Adhesion molecule involved in kidney function (CD151) Adhesion molecule, function in RBCs not known (CD108)

Antigens depressed in certain autoimmune and malignant conditions

Indian

IN (023)

11p13

IN (CD44)

Depressed in pregnancy, congenital dyserythropoietic anemia Renal disease

Ok Raph

Ok (024) RAPH (025) JMH (026)

19p13.3 11p15.5

OK (BSB) MER2 (CD151) JMH (SEMA L)

Oka MER2 [Raph]

JMH

15q24.1

JMH, JMHK, JMHL, JMKG, JMKM

Absent from PNH III RBCs


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International Society of Blood Transfusion Defined Blood Group Systems and Antigen Collections with Chromosome and Gene

Conventional Name I GLOB Gil RhAg

ISBT Symbol (No.) I (027) Globoside (028) GIL (029) RHAG (030)

Chromosome Location 6p24.2 3q26.1 9p13.3 6p11.21.1

ISBT Gene Name (ISGN If Different) IGNT P ( 3GALNT 1) GIL (AQP3) RHAG

Associated Antigens [Null Phenotype] I [I; i adult] P [P] GIL [GIL] Duclos, Osa, DSLK

Function of RBC Membrane Component (CD No.) Glycocalyx Glycocalyx Glycerol/water/urea transporter Possibly transports CO2, or NH3(CD241)

Disease Association

Congenital cataracts in Asians Receptor E. coli and parvovirus B19

Hemolytic anemia, hereditary stomatocytosis

Antigen Collections Cost COST (205) Ii Ii (207) Er ER (208) (Pk, LKE) (Lewis-like: Lec, Led) Vel Low incidence series High incidence series GLOB (209) Unnamed: (210) VEL (700) (901)

Csa, Csb I Era, Erb Pk, LKE Lec, Led

Vel, ABTI 14 2

CGD, chronic granulomatous disease; ISGN, International Society for Gene Nomenclature. SOURCE: Data from Daniels GL, Anstee DJ, Cartron J-P, et al,13 Issitt PD, Anstee DJ,5 and Reid ME, Lomas-Francis C.6,15

International Society of Blood Transfusion Defined Blood Group Systems and Antigen Collections with Chromosome and Gene

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