Diego Blood Group System

The Diego blood group system is the classi cation of human blood according to the
properties conferred by thepresence of an antigen designated Di. Although there
are21 known Diego antigens, the determination of an indi- viduals Diego blood type
is based on the antigens denoted Di a (identi ed in 1955) and Dib (identi ed in
1967). TheDiego blood group system is associated with a gene known as SLC4A1
(solute carrier family 4, anion exchanger, mem-ber 1). This gene encodes a
substance called band 3 protein, which is expressed on the surface of red blood
cells andplays a central role in mediating the transport of carbon dioxide in the
blood. Although mutations in SLC4A1 can give rise to diseases such as hereditary
ovalocytosis (a disease in which red blood cells are oval shaped, notround), a
number of other mutations result in the produc-tion of Diego antigens.The Dia
antigen is found in more than 35 percent of South American Indians and about 12
percent of peopleof Chinese and Japanese descent. Diego incompatibility of mother
and fetus can cause erythroblastosis fetalis.

2.Diego(DiandDi)
a
b

a.Usefulasaracialmarker,DiantigenbeingalmostentirelyconfinedtopopulationsofMongolian
a

origin,includingNativeAmericans.
b.OnehundredpercentofthewhitepopulationtypesasDi(ab+)

Diego blood group The erythrocyte antigen defined by anti-Da antibody was first
isolated in Venezuela, where it caused erythroblastosis fetalis. An antibody with
antithetical reactions, anti-Db, was discovered in 1967. The antigen system is
controlled by two alleles, Dia and Dib. The blood group antigens of this system are
caused by a single amino acid variation in the SLCA1 gene, which encodes the
bond-3 protein of the erythrocyte membrane. The Dia anti- gen is common in Native
Americans and Asians but is apparent- ly absent in whites. Two independent pairs of
antigens, Dia/Dib and Wra/Wrb, comprise the Diego system. Antibodies to Dia can
cause significant hemolysis, including hemolytic disease of the newborn

Transfusion reactions

Anti-Dia and anti-Dib are more commonly associated with HDN than transfusion reactions.
However, these antibodies are capable of causing immediate (9) and delayed hemolytic
transfusion reactions (2, 8).
Hemolytic disease of the newborn

HDN caused by Diego antibodies are more common in South East Asia and South America.
Anti-Dia is capable of causing moderate to severe HDN, and cases have been reported in Japan
(9), China (10, 11), and Poland (5).
Anti-Dib typically causes mild HDN. Cases have been reported in Japan (12), China (13), Poland
(6), and in a mother of South American descent (14).

Dia
(DI1)
Dib
(DI2)
Wra
(DI3)

Wrb
(DI4)
Wda
(DI5)
Rba
(DI6)

WARR
(DI7)
ELO
(DI8)
Wu
(DI9)

Bpa
(DI10)
Moa
(DI11)
Hga
(DI12)

Vga
(DI13)
Swa
(DI14)
BOW
(DI15)

NFLD
(DI16)
Jna
(DI17)
KREP
(DI18)

Tra
(DI19)
Fra
(DI20)
SW1
(DI21)

DISK
(DI22)