SBB Review

SBB Review Band 3 - macrocomplex of proteins including: -Rh -RhAG -LW (ICAM-4) -GPA -GPB -CD47 ATP source for RBCs - 90% from Glycolysis (Embden-Meyerhoff pathway) 10% from pentose phosphate pathway Methemoglobin Reductase Pathway - necessary to maintain heme iron in ferrous (Fe2+) state Deficiency causes accumulation of methemoglobin, nonfunctional from of Hgb Leubering Rapaport Shunt - permits accumulation of 2,3-DPG "Tense form" of Hgb - DEOXYGENATED widening of beta-chain lower affinity for O2 expels 2,3-DPG "Relaxed form" of Hgb - OXYGENATED beta-chain pulled together higher affinity for O2 expels 2,3-DPG "Shift Right" - higher P50 value decreased pH (increased H+ ion), acidosis increased 2,3-DPG higher temperature high pCO2 Seen in compensated anemia "Shift Left" - lower P50 value increased pH (decresed H+ ion), alkalosis decreasede 2,3-DPG lower temeratures low pCO2 Seen in storage of RBC Type 2 precursor - Gal-GlcNAc B1-4 Type 1 precursor - Gal-GlcNAc B1-3 Secreted (Se, Le) Type 2 H antigen transeferase & Sugar - FUT-1 Alpha-2-L-fucosyltransferase Fuctose alpha1-2 A antigen transferase & sugar - D-galactosaminetrasferase N-acetylgalactosamine alpha1-3 B antigen transferase & sugar - D-galacotsyltransferase galactose alpha1-3 Ax subgroup - weakly positive anti-A weakly positive anti-A,B Am subgroup - Negative anti-A Secretes A & H Ael subgroup - Negative anti-A Secretes H only Highest expression of H Lowest expression of H - O>A2>B>A2B>A1>A1B> H deficient Lewis transferase & Sugar - FUT-3 Fuctose alpha1-4 Type 1 H antigen transferase & sugar - FUT-2 Fuctose alpha1-2 anti-Le bh vs anti-Le bl - Anti-Le bh= reacts best with Leb & H are on rbc reacts best with group O and A2 does not react with A1 & B Anti-Le bl= reacts independently of amount of H on rbc best antisera to use when typing RBC autoanti-H, -HI is most common in... - A1 patients, have the least amount of H Elevated i antigen associated with... - cord cells reticulocytes in megaloblastic anemia leukemia stressed erythropoiesis I gene - IGnT adds GlcNAc to Gal starts branching/ multivalent anti-i associated with what disease - infectious mono autoanti-i, -I commonly seen in patients with.... - Mycoplasma pneumoniae infections Lymphoproliferative disorder (Waldenstrom macroglobinemia) P1K & P2K lack what gene/ antigen - B3GalNAct1 missing P antigen (also Gb5 & LKE) p lack what gene/ antigen - A4GALT missing P1/PX2 & Pk (also P, Gb5 &LKE) P1 substance derived from... - Hydaited cyst Bird eggs liver fluke autoanti-P associated with - Paroxysamal Cold Hemoglobinuria, PCH Donath Landsteiner Lutheran (E/DTT) - Enzyme= Decreased Lewis (E/DTT) - Enzyme= increased Diego (E/DTT) - unaffected by both Yt (E/DTT) - Enzyme= decreased DTT= decreased Scianna (E/DTT) - DTT= decreased Dombrock (E/DTT) - DTT= Sensitive...