:

Other Blood Group Systems

. .
-

2012
 RBCs
1. > 200 RBC Ags

2.

3. :
,


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 RBCs

:
-
:
IgM
:
IgG
 /

1. : 1.
,


2. :
2.


3. :

3.




1. 1. , , ,
., ,
:
, .
2.
2. : !!!

3. : 3. >10,000 daltons

4. 4.
: HLA
: : ABH, Le, Ii
P (Ag)
: Rh, M, N

()
RBC Vs. RBC

RBC :

(-D ..)

RBC :

(-A, -,

..)
 IgG

1. 1.

2. 2. : 37oC

3.
3.
4.
4. . IgG
C.
(C)

5. 5.
 IgM
1.
1.

2. 2. : 4-10oC

3. 3.
4. ( )
4. - IgM
C

5. 5.


IgM
.
! ;

ABO = .

.

-M, -N, ..
IgM .


= - ,
-



*
(
)

E Rh 16-40% 30% 4%
Kell (Kl) Kell 5-40% 9% 9%
D Rh 8-33% 85% 70%
c Rh 4-15% 80% 4%
Jk(a) Kidd 2-13% 77% 0.14%
Fy(a) Duffy 4-12% 63% 0.46%
C Rh 2-10% 70% 0.22%
e Rh 2-3% 98% 1%
Jk(b) Kidd 2% 72% 0.06%
S MNSs 1-2% 55% 0.08%
s MNSs <1% 89% 0.06%

1. : Abs
/ RBCs

HDN

2. M K : Abs
RBCs

3. (Cold reacting antibodies):

4. (Warm reacting antibodies):

37C
 RBCs

Lewis
I Kell ;

P
MNSs
Kell
Kidd
Duffy


 ;
Anti-

Anti-H

Anti-B
, , IgM
 Lewis
 Lewis
1. 1946 Mourat

2. Lea, Leb

3. Le ()
le (), Le/Le, Le/le, le/le
 Lewis

1. To Lewis

2. (Se/Se, Se/se), (se/se)

3. Le/Le, Le/le (Lewis+) & se/se = Lea

4. Le/Le, Le/le (Lewis+) & Se/Se, Se/se = Leb

5.
 Lewis

,
()
RBCs

2
Lewis substance
adheres to RBC
becoming an antigen

RBC

Le substance
in plasma
Le
genes Ags
 Lewis

Lewis Hh, Se, Le

le, h, se
Le Lea
Le, H, Se
Lea Leb. :

Le se H Le(a+b-)
Le Se H Le(a-b+)
le H se Le(a-b-)
le hh se Le(a-b-)


Phenotype White Black

Le a+b- 22% ---

Le a-b+ 72% ----

Le a-b- 6% 20%
 Lewis
Le(a-b-)

IgM ( , )



Le(a-b-)
;
Le Abs
Lewis
HDN
( )
Le(a-b-)
 Lewis
1. Anti-Le a, Anti-Le b, Anti-Lex
2.
3.
4. in vitro
5. Le a

Anti-Le a
Lea-b-


in vitro
in vivo
 Lewis
2. Anti-Le b
Anti-Lea

:
- Anti-LebH
- Anti-LebL


3. Anti-Lex

Lea Leb

 Lewis

1. Lewis

2. Lewis
Lewis

3. ( )

Lewis
 Abs ;

1.

2.
Lewis

 -Lewis - ;;;

IAT 37C

;;;

80% Le (a-)

30% Le (b-)


I i
RBCs
i


, ,


i (linear, ) I (branched,
) 18

--I (RT 4C)

--I
(Cold-reacting)
IgM


37C
37oC


1. Anti-I :
anti-IH

RBCs
Ag-H RBC
O RBCs
A RBCs ( Ag-A)
Cold Agglutinin Disease (similar to
PCH)
Mycoplasma
pneumoniae
2. Anti-I :

, ,


Anti-IA
Anti-IH
Anti-IP1


 - Adsorption

RBCs

 -I - ;;;

-I 37C

 P

1. 1927 Landsteiner g-P

2.

3. Ags P1, P, Pk, p

4. 5

5. Ags 2
1 ( 22)
( 6)
, ,
 To
ABO
P1 P2
P1: P1 P
P2: P
A2, P2 anti-P1

75% P1
 To 1

, (Cyst of a dog tapeworm)

1. Anti-P1
IgM



2. Anti-P

[paroxysmal cold hemoglobinuria
(PCH)]
PCH IgG auto-anti-P
( Hgb)
PCH : Donath-Landsteiner
antibody

3. Anti-pk Anti P + P1 + pk

Anti-pk

Anti-P + P1 + pk p

Anti-Tja

Anti-Tja (PP1Pk) isoimmunization. A case, a review of the literature

Haentiens-Verbeke et al., 1993
 -P -
;;;
-P 37C

;;;
20% P (-)
 Ss
 MNSs
1. 1927-1947 Landsteiner & Levine

2. Ags M, N, S, s, Su

3. Ags 29 S
s

4. RBCs = ,

5. 2 4

6. ,
Ags M, N
Ags S, s
 MNSs
5 :
M
N
S
s
U ( S & s )

M & N Glycophorin A
S & s U Glycophorin B

O Glycophorins
: RBCs

 MNSs

M M & N

Glycophorin A N
1 and 5

RBC

S
S & s
U
s
Glycophorin B 29

COOH end .. .5, 4, 3, 2, 1 (NH2 end)

 MNSs

M & N
, (M+N-) or (M-N+)

(M+N+)

(ficin, papain)
 -
 U (Su)
U
S & s

85% S-s-
U- ()

U-



Phenotypes Blacks (%) Whites

(%)
M+ 74 78
N+ 75 72
S+ 30.5 55
s+ 94 89
U+ 99 99.9

..

MNSs ;

Mk M, N, S, s
0.00064 0.064%
 - -
(dosage antibodies)
Anti-M anti-N
IgM ( IgG)

IgG, HDN ()

Dosage antibodies:

;

 -S, -s -U

(Clinically significant)
IgGs
RBCs HDN
Anti-U
S+ s+
S-s-
U-
<1%
()

Rhnull RBCs
Anti-Nf

 MNSs

Dosage antibodies
IgG

Anti-M IgM
( IgG)
Anti-N IgM

Anti-S IgG

Anti-s IgG

Anti-U IgG

MNSs

( )

-S IgM -s
IgG (HDN )
-MNS -
;;;

- 37C

- 37C

-S & -s

-U
U ( S-s-)

;;;

22% (-)

28% (-)

45% S (-)

11% s (-)

<1% ( ) U (-)


 ; Anti-C
Anti-c

Anti-E
Rhesus Anti-e
, , IgGs

Anti-D
!!!
Ag-d anti-d
 Kell
 Kell
Rh
2 (> 20 )
K (Kell), <9%
k (cellano), >90%
K k , 7 (7q33)

To K (2 D )

RBCs

Rh

 Kell
Kell:
Rh : C/c E/e
Kp
Kpa = ( 2%)
Kpb = (99.9%)
Js
Jsa (20% , 0.1% )
Jsb = (80-100%)
 Kell

Numeric Alpha Name Incidence

KEL 1 K Kell 10%
KEL 2 k Cellano 99.8%
KEL 3 Kpa Penny 2%
KEL 4 Kpb Rautenberg 99.9
KEL 6 Jsa Sutter Rare (19% Blacks)
KEL 7 Jsb Matthews 99.9%(99.8% Blacks
 Kell

Kell

sulfhydryl :
2-mercaptoethanol (2-ME)
Dithiothreitol (DTT)
2-aminoethylisothiouronium bromide (AET)
 Kellnull K0
Kell
Kx
, K0 -Ku
(Ku )
Kell

To


KEL
RBCs

(-5)

 K0

Molecular Basis of the Kell-null Phenotype Lung-Chih Yu et al., 2001

 McLeod

()
()


Duchene

 McLeod

The Kell blood group system: a review, MARSH AND REDMAN, 1989
 McLeod
Point mutations causing the McLeod phenotype Russo et al., 2002
 Kmod

Kmod

-5

Mutations that diminish expression of Kell

surface protein and lead to the Kmod RBC
phenotype, Lee et al., 2003
 Kell
IgG ( AHG)

(, )

Anti-K ( Kell )
k, Kpb, Jsb (
)

-

 Kx
Kell,

Kx
Kell

Kx K0
-Kell - ;;;

-K

-k k

-Kpb Kpb

- Jsb Jsb

-Ku Ko
 ;;;
0,2% +k -

0,01% Kp (a+b-)

Js (a+b-)

Ko
 Kidd
 Kidd
2
Jka Jkb (codominant alleles)

(%)
(%)
JkaJka Jk(a+b-) 26.3 51.1
JkaJkb Jk(a+b+) 50.3 40.8
JkbJkb Jk(a-b+) 23.4 8.1
JkJk Jk(a-b-)
 Kidd

 Kidd
-Jka -Jkb
IgG


(HTR)
(HDN)



 Kidd
Anti-Jk3
Jk(a-b-)

()
 -Kidd -
;;;
-Jka -Jkb Jka Jkb

Anti-Jk3 Jk(a-b-)

;;;

24% Jka-

27% Jkb-
 Duffy
 Duffy
(codominant alleles):
Fya Fyb

Fy(a+b-) 9 17
Fy(a+b+) 1 49
Fy(a-b+) 22 34
Fy(a-b-) 68
 Duffy A
IgG

(
)

-Duffy - ;;;
-Fya -Fyb Fya Fyb

-Fy3 () Fy(a-b-)

;;;

32% Fy(a-)

20% Fy(b-)


 Lutheran
2 ( ) codominant alleles:
Lua Lub

92% Lub , Lu(a-b+)

Lu(a-b-)
The Lutheran glycoprotein: a
multifunctional adhesion
receptor, Eyler and Telen, 2006
 Lutheran
Anti-Lua
IgM IgG




Anti-Lub
, Lub = Ag
IgG
(
HDN)
-Lutheran- ;;;

-Lua

-Lub Lub

;;;
92% Lu (a-)
 Bg
Bg :

Bga

Bgb

Bgc

HLA

 Sda

,





 Xg

89% Xga
66% (carry only one X)

Xga Xg
XgaXga, XgaXg, XgXg
: Xg(a+) Xg(a-)
Xg(a+)

IgG
 Chido/Rogers

Rg + Ch +





Gerbich
7

IgG

Scianna
Sc:1 - 100% Sc:2 - 0.3% Sc:3 - 100%



Colton
: Coa -99.7% Cob -10.7% Co3 -100%
Colton null phenotype

IgG

Cromer
10 (7 )



Cartwright
Yta - 99.8% Ytb - 0.2%
IgGs

Dombrock
Doa - 57% Dob - 83%
Holly, Gregory,
Joseph

HTR (Hemolytic Transfusion Reactions)?
HDN?


IN
Ina Inb
Ina
Ina
HTR

Knops
5

HTLA
 HTLA

High Titer Low Avidity (HTLA)

HDN HTR

 (IgM)
Anti-Lea
Anti-Leb
Anti-I
;;;
Anti-P1
Anti-M
Anti-A, -B, -H
Anti-N

LIiPMABHN


 (IgG)

Rh antibodies
Kell
Duffy
Kidd
S,s
 :

Papain, bromelin, ficin, trypsin

Kidd Fya and Fyb

Rh M, N
Lewis S, s
I
P
 :
Kidds Duffy (Rh)
M&Ns

M&Ns
M&Ns

anti-Jka, -Jkb, -Fya, -Fyb, anti-C, -c, -E, -e (no D), -M, -N, -S, -s
MNSs
Kidd Duffy Rh

adapted from Clinical Laboratory Science Review: A Bottom Line Approach (3rd Edition)