Other Blood Group Systems

By Dr. Christina ThompsonTexas A&M University-

Corpus Christi

LEWIS SYSTEM

A serum antigen secondarily absorbed to the red cells

Le gene produces Lea

Secretors change the Lea to Leb

Le may also modify the A antigen review the relationship to ABO precursors

Lewis Red Cell Phenotypes

Genes Lewis Red Cell Phenotype

Le Se Lea- Leb+

Le se Lea+ Leb-

lele Lea- Leb-

le se Lea- Leb- Lec+

le Se Lea- Leb- Lec- Led+

Development of Antigens

Newborns born Le a-b-

If Le and Se – 2 weeks to 6 months Le a+

– then Le a+b+

– then Le a-b+

During pregnancy, antigens become weaker

Phenotype Frequencies

Phenotype White Black

Le a+b- 22% ---

Le a-b+ 72% ----

Le a-b- 6% 20%

Lewis Antibodies

Anti-Le a, Anti-Le b, Anti-Lex

Most react at room temperature or below -

Often fix complement Some in vitro hemolysis Le a may cause HTR

Lewis Antibodies

Anti-Le a

Found in Lea-b- secretors best room temperature or below - some

at ICT and enzymes Often fix complement Some in vitro hemolysis Le a may cause HTR

Lewis Antibodies

Anti-Le b

Often found with Anti-Lea

Most react at room temperature or below

Two types - Anti-LebH and Anti-LebL

Rare cause of HTR

Lewis Antibodies

Anti-Lex

Most react at room temperature or below -

Reacts with both Lea and Leb as a single antibody

Lewis Antibodies

Special Problems in the Blood Bank– Lewis antigens may be weaker during

pregnancy and women produce antibodies– Can neutralize Lewis antibodies with Lewis

plasma– Pregnant woman with room temperature

antibodies, neutralize with Lewis antigen when testing for HDN antibodies

I Blood Group

Two antigens I and i I antigen present on almost all healthy

adults Rare adults that are I negative -

spectrum on page 175 I antigen varies in strength on adult cells

I Blood Group

Newborns do not have much I antigen Newborns have i antigen At about 18 months the i is replaced

with I Some transitional antigens

I Blood Group

I substance can be found in saliva and human milk and on lymphocytes and platelets

During disease, the I antigens may alter

I Blood Group

Antibodies Anti-I anti-i– Anti-I

usually reacts at room temperature, saline or below

often attaches complement doesn’t cause hemolysis unless it reacts at 37oC Can be found in almost all sera in low titers and

titers increase during some diseases (viral infections - syphilis - atypical pneumonia)

COLD AUTOAGGLUTIN

I Blood Group

Antibodies Anti-I anti-i– Anti-i

rare antibody occurs in patients with infectious mononucleosis, cirrhosis, myeloid leukemia, reticulosis

I Blood Group Antibodies

– Other combination antibodies have been found (IA, IH, IP1, etc.) pp. 176 - 177

– ENZYMES ENHANCE ACTIVITY

– ABSORBTION IS USED TO TEST FOR OTHER MORE IMPORTANT ANTIBODIES

AutoabsorptionAutoabsorption

P Blood Group Discovered in 1927 by Landsteiner Antigens P1 P p pk Luke

– Luke antigen and disease association - page 173

P Blood Group

Antibodies Anti-P1 Anti-P Anti-pk Anti- P + P1 + pk

– Anti-P1 Usually IgM reacts at room temperature and

saline May attach complement rarely a problem with transfusion easily inhibited with P1 substance

P Blood Group

Antibodies Anti-P1 Anti-P Anti-pk Anti- P + P1 + pk

– Anti-P

found in sera from pk individuals - an IgM hemolytic antibody that is clinically significant

also found as an IgG biphasic antibody in parozysmal cold hemoglobinuria called Donath-Landsteiner antibody

P Blood Group

Antibodies Anti-P1 Anti-P Anti-pk Anti- P + P1 + pk

– Anti-pk and Anti P + P1 + pk

Anti-pk has only been found as part of other antibodies

Anti-P + P1 + pk found in p individuals - formerly called Anti-Tja and very hemolytic

Duffy Blood Group

Discovered in early 1950’s Fy antigen locus on chromosome 1 with Rh

locus Antigens

codominant inheritance– Fya Fyb Fyx

– Others Fy3 Fy 4 Fy5 Fy6 Fs - (page 185)

Duffy Blood Group

Duffy Blood Group Fya-b- appear to provide some protection

from P.vivax infection Antibodies Anti-Fya Anti-Fyb

– Usually AHG reaction - IgG– destroyed by enzymes– Rare examples of antibodies to other antigens

(Anti-Fy 3, Anti-Fy4, Anti-Fy5) and those reactions are not destroyed by enzymes

– Cause HTR and HDN

Kell Blood Group Many antigens in this system and has

been given a numerical nomenclature Refer to table 8-8

Six most important 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 Blood Group

Most common gene complexes

Kell Blood Group

Mc Leod syndrome– Reduced expression of Kell antigens– association with hemolytic anemia and

chronic granulomatous disease – genetics and antigen page 181

Kell Blood Group

Antibodies

– Usually IgG and require AHG – rare reaction in saline– common antibodies– implicated in HTR and HDN– Anti-K is a very common antibody

MNSs Blood Group

Many antigens in this system and some are alleles to the four common antigens

M N S s Association with GPA and GPB Four gene complexes

MS Ms NS Ns Other alleles Mg, Mk, Mc, Mr, Mz, Mv, Na, T1m,

Sj, S2, some quantitative differences

MNSs Blood Group Phenotypes

MNSs Blood Group

U antigen is absent or reduced on S-s- Other antigens - page 165 Mi - abnormal forms of Ss glycoprotein En(a-) absence of MN glycoprotein Disease association Page 170

MNSs Blood Group Antibodies Anti-M and Anti-N

– Usually room temperature– IgM saline reaction– Dosage (antibodies react better with

homozygous cells)– Destroyed by enzymes – Possible HDN and HTR if reaction at AHG– Anti-Nf found in dialysis patients

MNSs Blood Group

Antibodies Anti-S

– Usually igM and room temperature although some at AHG

– destroyed by enzymes– Rare HTR and HDN

MNSs Blood Group Antibodies Anti-s and anti-U

– Usually IgG and AHG– Not destroyed by enzymes– HTR and HDN– Anti-U found as warm autoantibody and

does not react well with Rh null cells– Other antibodies rarely detected but not

uncommon (ex. anti-Mg common antibody)

Kidd Blood Group

Discovered in the 1950s Two antigens Jka Jkb

Kidd Blood Group

Antibodies - Anti-Jka and Anti-Jkb

– Usually IgG and require AHG– bind complement– enhanced by enzymes– implicated in HDN and HTR– Seldom potent and deteriorate rapidly– Classic delayed HTR

Kidd Blood Group

Antibodies Anti-Jk3

– found in some Jka-b- individuals– reacts with Jka and Jkb

Lutheran Blood Group

Two antigens Lua (8%) Lub (99%)– Other antigens Table 8-12

Important blood group that demonstrates multiple methods for inheritance of the null cell type

Lu a-b- inheritance– InLu dominate inhibitor gene– lulu recessive lack of Lu gene– sex linked inhibitor gene

Lutheran Blood Group Antibodies

– Anti-Lua - not common - reacts in saline but can be IgG and require AHG - gives a (mf) agglutination - unclear about HTR & HDN

– Anti-Lub - rare - mostly IgG and requires AHG - probable HTR and HDN

– Anti-Luab (Anti-Lu3 ) - reacts with all but

Lu a-b- of the recessive type – Other antibodies react with rare Lu phenotypes

found on Lua-b- (page 192/3)

Other Blood Groups Diego - Dia Dib Wra Wrb 3 others

– Dia found in Chippawah Native Americans and Japanese and Chinese

– uncommon antibodies - AHG reaction and important in HTR and HDN

– Wra is a low incidence antigen and Wrb is a high incidence antigen

– anti-Wra is a fairly common antibody - IgM or IgG

Other Blood Groups

Chido/Rogers– Nine antigens - all normal individuals are

either Rg + or Ch +– HTLA - use plasma inhibition– Determinants on C4 molecule and linked to

HLA -

Other Blood Groups

Xg– sex-linked inheritance

Xga positive Male - 66% Female - 89%

– uncommon antibody - AHG reaction and destroyed by enzymes - HTR and HDN?

Other Blood Groups

Gerbich– system with at least 3 high incidence

antigens and 4 low incidence antigens– Antibodies usually IgG which require AHG

and clinically significant Scianna

– Sc:1 - 100% Sc:2 - 0.3% Sc:3 - 100%– Antibodies are rare

Other Blood Groups Colton

– antigens: Coa -99.7% Cob -10.7% Co3 -100%– the null phenotype has been found and

associated with genetic abnormality and anemia

– antibodies IgG and clinically significant Cromer

– consists of 7 high incidence antigens and three low incidence antigens

– antibodies probably clinically significant

Other Blood Groups

Cartwright– antigens Yta - 99.8% Ytb - 0.2%– Usually IgG and AHG ?HDN and HTR?

Dombrock– antigens Doa - 57% Dob - 83%– additional antigens added Holly, Gregory,

and Joseph – Uncommon antibodies HTR and ?HDN?

Other Blood Groups

IN– Ina Inb

– Ina Iranian and Arabs– Enzyme destroyed - Ina HTR

Knops– five antigens– depressed in some diseases– HTLA

Other Antigens

High incidence– Vel, Lan, August, Jacobs, Sid, Wra

Low incidence– too numerous to mention

Bg - HLA antigens that coat red cells

Other Blood Group Systems

By Dr. Christina ThompsonTexas A&M University-

Corpus Christi