Kell blood group system genotyping in blood
donors from Tehran transfusion center
• Supervisor : Dr Kamran Moosavi
• Consultant : Dr Majid Shahabi
• Presenter : Maryam Pourshadloo
Significance of this survey
• After ABO and Rh ; most immunogenic ; most clinically significant in
transfusion medicine
• 32 antigen : high frequency and low frequency
• Glycoprotein hemolytic disease of the fetus and newborn (HDFN )
immediate and delayed hemolytic transfusion
reaction ( HTRs )
• Individuals with Knull phenotype require Knull phenotype for
transfusion
history
• Discovered in 1946
• Mrs. Kelleher anti- kell antibodies HDN
• Until now 32 antigens
• Different frequency in different populations
Kell antigen system
• group of antigens on the human red blood cell surface
• important determinants of blood type
• targets for alloimmune diseases and autoimmune
Gene
• on chromosome 7, at 7q33
• 19 exons
• more than 21 kbp of genomic DNA
• highly polymorphic SNP single amino acid substation
Kell 1 and Kell2 N 518
aa 193
Protein
• type II trans membrane glycoprotein
• 732 amino acids 47 N-terminal cytoplasmic
20 transmembran
665 C-terminal extracellular
• Extracellular domain 5 N- glycosylation sites
15 cysteine residues disulphide bound and
folding
Protein
• Covalently links via a single disulfide bond to the XK membrane
protein
• XK gene is on the short arm of the X chromosome
• All but two ( Jsa and JSb ) of the Kell antigens are localized in the
N-terminal half of the protein before residue 550
antigens
five sets of high-incidence and low-incidence antigens
• K and k
• Kpa, Kpb, and Kpc
• Jsa and Jsb
• K11 and KI7
• K14 and K24
Null phenotype
• K0 in which RBCs lack all Kell antigens
• healthy but produce anti-Ku when they encounter RBCs that do
express Kell antigens
• K0 individuals transfused with K0 blood products
McLeod syndrome
• Absence of XK
• Kell antigens weakly expressed
• Abnormal RBC with spiky projections
• chronic granulomatous disease (CGD)
• neurological defects
Kell antibodies
• K antigen most immunogenic Transfusion reactions
Hemolytic disease of the fetus and
newborn
• usually of the antibody class IgG ; predominantly IgG1
• Transfusion reaction ; HDN ; neonatal anemia
• Bacterial infections ( mycobacterium ) IgM
Kell antibodies
• K antigen appears on fetal red cells earlier than Rh proteins
• Anti-K facilitates phagocytosis before the cells produce hemoglobin
• Anemia is severe ; anti-K cause a suppression of erythropoiesis rather
than the immune destruction that occurs with anti-D
Propose of this survey
Recognizing Kell blood group system genotype in blood donors from
Tehran transfusion center

Proposal

  • 2.
    Kell blood groupsystem genotyping in blood donors from Tehran transfusion center • Supervisor : Dr Kamran Moosavi • Consultant : Dr Majid Shahabi • Presenter : Maryam Pourshadloo
  • 3.
    Significance of thissurvey • After ABO and Rh ; most immunogenic ; most clinically significant in transfusion medicine • 32 antigen : high frequency and low frequency • Glycoprotein hemolytic disease of the fetus and newborn (HDFN ) immediate and delayed hemolytic transfusion reaction ( HTRs ) • Individuals with Knull phenotype require Knull phenotype for transfusion
  • 4.
    history • Discovered in1946 • Mrs. Kelleher anti- kell antibodies HDN • Until now 32 antigens • Different frequency in different populations
  • 5.
    Kell antigen system •group of antigens on the human red blood cell surface • important determinants of blood type • targets for alloimmune diseases and autoimmune
  • 6.
    Gene • on chromosome7, at 7q33 • 19 exons • more than 21 kbp of genomic DNA • highly polymorphic SNP single amino acid substation Kell 1 and Kell2 N 518 aa 193
  • 7.
    Protein • type IItrans membrane glycoprotein • 732 amino acids 47 N-terminal cytoplasmic 20 transmembran 665 C-terminal extracellular • Extracellular domain 5 N- glycosylation sites 15 cysteine residues disulphide bound and folding
  • 8.
    Protein • Covalently linksvia a single disulfide bond to the XK membrane protein • XK gene is on the short arm of the X chromosome • All but two ( Jsa and JSb ) of the Kell antigens are localized in the N-terminal half of the protein before residue 550
  • 10.
    antigens five sets ofhigh-incidence and low-incidence antigens • K and k • Kpa, Kpb, and Kpc • Jsa and Jsb • K11 and KI7 • K14 and K24
  • 11.
    Null phenotype • K0in which RBCs lack all Kell antigens • healthy but produce anti-Ku when they encounter RBCs that do express Kell antigens • K0 individuals transfused with K0 blood products
  • 12.
    McLeod syndrome • Absenceof XK • Kell antigens weakly expressed • Abnormal RBC with spiky projections • chronic granulomatous disease (CGD) • neurological defects
  • 13.
    Kell antibodies • Kantigen most immunogenic Transfusion reactions Hemolytic disease of the fetus and newborn • usually of the antibody class IgG ; predominantly IgG1 • Transfusion reaction ; HDN ; neonatal anemia • Bacterial infections ( mycobacterium ) IgM
  • 14.
    Kell antibodies • Kantigen appears on fetal red cells earlier than Rh proteins • Anti-K facilitates phagocytosis before the cells produce hemoglobin • Anemia is severe ; anti-K cause a suppression of erythropoiesis rather than the immune destruction that occurs with anti-D
  • 15.
    Propose of thissurvey Recognizing Kell blood group system genotype in blood donors from Tehran transfusion center