Other Blood Group Systems By Dr. Christina Thompson Texas A&M University- Corpus Christi

LEWIS SYSTEM n n A serum antigen secondarily absorbed to the red cells n n Le gene produces Le a n n Secretors change the Le a to Le b n n Le may also modify the A antigen review the relationship to ABO precursors

Lewis Red Cell Phenotypes Genes Lewis Red Cell Phenotype Le Se Le a- Le b+ Le se Le a+ Le b- lele Le a- Le b- le seLe a- Le b- Le c+ le SeLe a- Le b- Le c- Le d+

Development of Antigens n n Newborns born Le a-b- n n 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 n n Anti-Le a, Anti-Le b, Anti-Le x n n Most react at room temperature or below - n n Often fix complement n n Some in vitro hemolysis n n Le a may cause HTR

Lewis Antibodies n n Anti-Le a n n Found in Le a-b- secretors n n best room temperature or below - some at ICT and enzymes n n Often fix complement n n Some in vitro hemolysis n n Le a may cause HTR

Lewis Antibodies n n Anti-Le b n n Often found with Anti-Le a n n Most react at room temperature or below n n Two types - Anti-Le bH and Anti-Le bL n n Rare cause of HTR

Lewis Antibodies n n Anti-Le x n n Most react at room temperature or below - n n Reacts with both Le a and Le b as a single antibody

Lewis Antibodies n n 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 n n Two antigens I and i n n I antigen present on almost all healthy adults n n Rare adults that are I negative - spectrum on page 175 n n I antigen varies in strength on adult cells

I Blood Group n n Newborns do not have much I antigen n n Newborns have i antigen n n At about 18 months the i is replaced with I n n Some transitional antigens

I Blood Group n n I substance can be found in saliva and human milk and on lymphocytes and platelets n n During disease, the I antigens may alter

I Blood Group n n Antibodies Anti-I anti-i – –Anti-I n n usually reacts at room temperature, saline or below n n often attaches complement n n doesn’t cause hemolysis unless it reacts at 37 o C n n Can be found in almost all sera in low titers and titers increase during some diseases (viral infections - syphilis - atypical pneumonia) n n COLD AUTOAGGLUTIN

I Blood Group n n Antibodies Anti-I anti-i – –Anti-i n n rare antibody occurs in patients with infectious mononucleosis, cirrhosis, myeloid leukemia, reticulosis

I Blood Group n n Antibodies – –Other combination antibodies have been found (IA, IH, IP 1, etc.) pp – –ENZYMES ENHANCE ACTIVITY – –ABSORBTION IS USED TO TEST FOR OTHER MORE IMPORTANT ANTIBODIES

Autoabsorption

P Blood Group n n Discovered in 1927 by Landsteiner n n Antigens P 1 P p p k Luke – –Luke antigen and disease association - page 173

P Blood Group n n Antibodies Anti-P 1 Anti-P Anti-p k Anti- P + P 1 + p k – –Anti-P 1 n n Usually IgM reacts at room temperature and saline n n May attach complement n n rarely a problem with transfusion n n easily inhibited with P 1 substance

P Blood Group n n Antibodies Anti-P 1 Anti-P Anti-p k Anti- P + P 1 + p k – –Anti-P n n found in sera from p k individuals - an IgM hemolytic antibody that is clinically significant n n also found as an IgG biphasic antibody in parozysmal cold hemoglobinuria called Donath- Landsteiner antibody

P Blood Group n n Antibodies Anti-P 1 Anti-P Anti-p k Anti- P + P 1 + p k – –Anti-p k and Anti P + P 1 + p k n n Anti-p k has only been found as part of other antibodies n n Anti-P + P 1 + p k found in p individuals - formerly called Anti-Tj a and very hemolytic

Duffy Blood Group n n Discovered in early 1950’s n n Fy antigen locus on chromosome 1 with Rh locus n n Antigens codominant inheritance – – Fy a Fy b Fy x – –Others Fy 3 Fy 4 Fy 5 Fy 6 Fs - (page 185)

Duffy Blood Group nn nn

n n Fy a-b- appear to provide some protection from P.vivax infection n n Antibodies Anti-Fy a Anti-Fy b – –Usually AHG reaction - IgG – –destroyed by enzymes – –Rare examples of antibodies to other antigens (Anti-Fy 3, Anti-Fy 4, Anti-Fy 5 ) and those reactions are not destroyed by enzymes – –Cause HTR and HDN

Kell Blood Group n n Many antigens in this system and has been given a numerical nomenclature Refer to table 8-8 n n Six most important Numeric Alpha Name Incidence KEL 1 K Kell 10% KEL 2 k Cellano 99.8% KEL 3 Kp a Penny 2% KEL 4 Kp b Rautenberg 99.9 KEL 6 Js a Sutter Rare (19% Blacks) KEL 7 Js b Matthews 99.9%(99.8% Blacks

Kell Blood Group n n Most common gene complexes

Kell Blood Group n n Mc Leod syndrome – –Reduced expression of Kell antigens – –association with hemolytic anemia and chronic granulomatous disease – –genetics and antigen page 181

Kell Blood Group n n 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 n n Many antigens in this system and some are alleles to the four common antigens n n M N S s n n Association with GPA and GPB n n Four gene complexes MS Ms NS Ns n n Other alleles M g, M k, M c, M r, M z, M v, N a, T 1 m, Sj, S 2, some quantitative differences

MNSs Blood Group n n Phenotypes

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

MNSs Blood Group n n Antibodies n n 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-N f found in dialysis patients

MNSs Blood Group n n Antibodies n n Anti-S – –Usually igM and room temperature although some at AHG – –destroyed by enzymes – –Rare HTR and HDN

MNSs Blood Group n n Antibodies n n 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-M g common antibody)

Kidd Blood Group n n Discovered in the 1950s n n Two antigens Jk a Jk b

Kidd Blood Group n n Antibodies - Anti-Jk a and Anti-Jk b – –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 n n Antibodies n n Anti-Jk 3 – –found in some Jk a-b- individuals – –reacts with Jk a and Jk b

Lutheran Blood Group n n Two antigens Lu a (8%) Lu b (99%) – –Other antigens Table 8-12 n n Important blood group that demonstrates multiple methods for inheritance of the null cell type n n Lu a-b- inheritance – –InLu dominate inhibitor gene – –lulu recessive lack of Lu gene – –sex linked inhibitor gene

Lutheran Blood Group n n Antibodies – –Anti-Lu a - not common - reacts in saline but can be IgG and require AHG - gives a (mf) agglutination - unclear about HTR & HDN – –Anti-Lu b - rare - mostly IgG and requires AHG - probable HTR and HDN – –Anti-Lu ab (Anti-Lu 3 ) - reacts with all but Lu a-b- of the recessive type – –Other antibodies react with rare Lu phenotypes found on Lu a-b- (page 192/3)

Other Blood Groups n n Diego - Di a Di b Wr a Wr b 3 others – – Di a found in Chippawah Native Americans and Japanese and Chinese – –uncommon antibodies - AHG reaction and important in HTR and HDN – – Wr a is a low incidence antigen and Wr b is a high incidence antigen – –anti-Wr a is a fairly common antibody - IgM or IgG

Other Blood Groups n n 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 n n Xg – –sex-linked inheritance n n Xg a positive Male - 66% Female - 89% – –uncommon antibody - AHG reaction and destroyed by enzymes - HTR and HDN?

Other Blood Groups n n Gerbich – –system with at least 3 high incidence antigens and 4 low incidence antigens – –Antibodies usually IgG which require AHG and clinically significant n n Scianna – –Sc: % Sc: % Sc: % – –Antibodies are rare

Other Blood Groups n n Colton – –antigens: Co a -99.7% Co b -10.7% Co3 -100% – – the null phenotype has been found and associated with genetic abnormality and anemia – – antibodies IgG and clinically significant n n Cromer – – consists of 7 high incidence antigens and three low incidence antigens – – antibodies probably clinically significant

Other Blood Groups n n Cartwright – –antigens Yt a % Yt b - 0.2% – –Usually IgG and AHG ?HDN and HTR? n n Dombrock – – antigens Do a - 57% Do b - 83% – – additional antigens added Holly, Gregory, and Joseph – – Uncommon antibodies HTR and ?HDN?

Other Blood Groups n n IN – –In a In b – –In a Iranian and Arabs – –Enzyme destroyed - In a HTR n n Knops – –five antigens – –depressed in some diseases – –HTLA

Other Antigens n n High incidence – –Vel, Lan, August, Jacobs, Sid, Wr a n n Low incidence – –too numerous to mention n n Bg - HLA antigens that coat red cells

Other Blood Group Systems By Dr. Christina Thompson Texas A&M University- Corpus Christi