BLOOD GROUP SYSTEMS

KEM Hospital mumbai

BLOOD GROUP ANTIGENS

 Most genes on autosomal chromosomes & follow Mendelian rules of inheritance.  Majority demonstrate co-dominance (heterozygotes express both gene products).  Macro molecular glycoproteins & lipoproteins- potent immunogens.  A,B,D followed by K,Fy

BLOOD GROUP ANTIBODIES

Usually IgG or IgM ; rarely IgA. Alloantibody:- reacts with foreign Ag not present on patients own RBC. Autoantibody Naturally occurring:- antigenic stimulus unknown Immune stimulated

COMPLEMENT SYSTEM
 Usually Classical pathway.  Intravascular Hemolysis:- Abs against ABO,rarely other IgM bld grp Abs & IgG Abs of Kidd- terminal membrane attack complex ( C5b6789 ).  Extravascular:- IgG via RE system  Absence of C:-Phagocytic cells via F receptor

INTERNATIONAL SOCIETY OF BLOOD TRANSFUSION

 Over 700 RBCs Ags are recognized and are organized into 29 blood group systems.  High frequency/ Public Ags:- expressed by most donors  Rare / Private Ags

ABO SYSTEM (ISBT 001)

 3 Ags : A,B & H  4 Phenotypes A,B,AB & O.  Weak phenotypes:- A1 &A2  B(A):-Inherited AD, trace A Ag expression on grp B RBC.  Acquired B:-enzymatic deacetylation of grp A Ag to form a B-like Ag on RBC,seen in bact infection & Ca.

ABO SYSTEM
ANTIGEN ON RBC ANTIBODY IN PLASMA/ SERUM BLOOD GROUP

A B AB None

Anti B Anti A None Anti A and Anti B

A B AB O

BOMBAY BLOOD GROUP

 BOMBAY & PARA BOMBAY:- Absence of all ABH Ags, (Oh)phenotype.  Frequency :- 1 in 7600  Subgroups of A & AB:-A1,A2,A1B & A2B, react with anti A1 Lectin or human antiA1.

ABO TYPING
 ICMR:- blood- agglutination, saliva- inhibition.

MNS SYSTEM (ISBT 002)

 Expressed only on RBCs on Glycophorin A,B &E-major sialomucin.  3 phenotypes M,MN & N.  3 phenotypes S,Ss & s.  Abs against M&N:-naturally occurring IgM.  Abs against S,s&U:-IgG,Immune stimulated,reactive at 37C.  P.falci adhere via sialic acid to RBC, so glycophorin deficient persons are resistant

P SYSTEM (ISBT 003)

 3 Ags: P, Pk & P1 (glycosphingolipids)  P1 only on RBC whereas Pk & P also on lymphocytes, plasma, uroepithelium, fibroblasts & synovium.  Anti P1:-IgM ,naturally occurring, in hydatid cyst& liver flukes.  Anti P & antiP,P1,Pk:-potent hemolysins, IgM or IgM + IgG3.

BIOLOGICAL ROLE OF P Ag
 Pk:- IF- & MHC II receptor, modulate cell signaling.  P:- cell differentiation & neoplasia.  Pk:-marker of apoptosis in germinal centre of B cells, Burkitt Lymphoma & Lymphocytic leukemia.  Receptor for Parvo B19,shiga toxin ,EHEC,P fimbriae of E.coli,Str suis & P.aeruginosa.

P PHENOTYPES
PHENOTYPE RBC Ag ANTIBOBY

P P2 P1k P2k p

P1,P,Pk Pk,P P1,Pk Pk -

ANTI P1 ANTI P ANTI P ANTI P,P1,Pk (ANTI Tja)

RH SYSTEM (ISBT 004)

 RH DEFICIENCY SYNDROME:- lack of all Rh Ags.Also lack Fy5 & LW Ags & markedly reduced S/s & U Ags.  Spheroctes & Stomatocytes.  osmotic fragility & RBC survival  Mild hemolytic anemia.  Transfusion difficult.

LUTHERAN (ISBT 005)

 Lu & Lu b Ags,rare in Indians.  Abs not often encountered.  High affinity for Laminin.  Over expression in ovarian ca &other catumor cell adhesion n metastasis.  Sickle cell dis:- Lu on retic & sickle cellsvaso-occlusive crises.

KELL & KX (ISBT 006 & 019)

 Gene on chromosome 7, shares homology with CALLA, ECE-1 & neutral endopeptidases.  Abs:-HTR& HDN  McLeod Syndrome:-absence of XK protein on RBC, brain, heart & skeletal muscles.  Associated chronic granulomatous dis

LEWIS (ISBT 007)

 Ags :-Le & Le b, passively adsorbed.  Also on plasma,saliva,platelets, lymphocytes,endothelium & uroepi.  Abs:-IgM, naturally occurring.  H.pylori binds to Le, aberrant expression in GIT & Uroepi - tumor mets.  Lewis null:-recurrent vaginal candiasis & UTI , also at risk for heart diseases.

DUFFY (ISBT 008)

 Ag on DARC gene (duffy antigen receptor for chemokines) on RBC, purkinge cell, post capillary venule endothelial cells, renal glomeruli endo cell, vasa recta , thyroid & pul capillaries.  Abs:-HTR & HDN ,IgG ,37C  DARC P.vivax, retic from Fy nullresistant

KIDD (ISBT 009)

 Ags:- Jka Jkb  Abs:-HTR, IgG  Jk facilitated transport of urea, maintain RBC osmotic stability.

DIEGO (ISBT 010)

 21 Ags , on Anion Exchange protein-1  No null phenotype.  Gas transport & acid base equilibrium  Stability of RBC membrane.

CARTWRIGHT (ISBT 011)

 Ags:- Yta & Ytb  Ags located on enzyme Acetyl cholinesterase.  Abs:-benign, IgG, immune stimulated, may RBC survival.

XG (ISBT 012)
 One AG:- Xg + or on X chromosome.  Higher frequency in women.  Specific for RBC.  Abs:- naturally occurring or immune stimulated, IgG.

COLTON (ISBT 015)

 Ags: Co a & Co b on aquaporin 1  Abs:- RBC survival, HTR & HDN , IgG ,immune stimulated.  AQ-1 major water channel on RBC & kidney.

LANSTEINER WIENER (ISBT 016)

 Ags:- LWa & LWb (glycoproteins)  Abs:- IgG, benign.  LW expression in sickle cell pts & RBC senescence.

CHIDO RODGERS (ISBT 017)

 10 Ags on C4d fragment of complement.  Abs benign.  C4 deficiency:-autoimmune dis & susceptible to bact meningitis.

GERBICH (ISBT 020)

 7Ags on glycophorin C & D (GYP C&D)  Abs:-HTR.  Glycophorin anchor membrane to cytoskeleton.  Ge null- marked elliptocytosis.  GYP C&D bind influenza virus & P.falci.

CROMER (ISBT 021)

 13 Ags on DAF CD 55  Abs:-HTR, RBC survival, IgG, immune stimulated.  CD55 protects cells from C & is uroepi & intestinal E.coli.  Null phenotype-chronic GI dis, protein losing enteropathy.  CD55 absent on PNH III RBC.

KNOOPS (ISBT 022)

 Complement receptor 1 (CR-1, CD 35)  Abs not significant.  CR 1binds C3b/C4b immune complexes, promoting their degradation.  Enhances phagocytosis in Leigonella,Leishmaniasis & mycobacteria.  CR-1 binds P.falci with rosette formation.

INDIAN (ISBT 023)

 Ags:- I & I  Abs:-IgM at room temp.  Auto Abs to I common & low titre cold agglutinins.  Cold AIHA:-malignancy, infectious mononucleosis.

OTHER SYSTEMS
 SCIANNA (ISBT O13)  DOMBROCK (ISBT 014)  HH (ISBT 018)  OK (ISBT 024)  RAPH (ISBT 025)  JOHN MLTON HAGEN (ISBT 026)  GLOBOSIDE (ISBT 028)  GIL (ISBT 029)