SBB Exam Review
Band 3 macrocomplex of proteins including:
-Rh
-RhAG
-LW (ICAM-4)
-GPA
-GPB
-CD47
Ankryin mediates interaction between Band 3 and underlying rbc skeleton
attaches to spectrin through interaction with protein 4.2
Band 4.1 mediates interactions between GPC and underlying rbc skeleton
Adducin interacts with dimeric Band 3 (associated with GPC)
Important for maintaining membrane stability
Spectrin major component of underlying skeleton
consists of 2 subunits: alpha & beta
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
Allows rbc to regulate O2 transport during hypoxia
Embden-Meyerhoff Pathway Whole anaerobic glycolysis pathway taken by rbc
generates 90% of ATP needed for rbc
"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
, SBB Exam Review
Compensated Anemia physiologic reactions Increased:
-Cardiac output (heart rate, stroke volume)
- Blood flow (vasodilation, shunting)
- O2 extraction
- O2- Hgb affinity (shift right)
Dyserythropoiesis HEMPAS, sideroblastic anemia, megaloblastic anemia
Altered erythropoesis quantitatively and qualitatively
increased i
associated with Hypoglycosylation
Myeloproliferative Disease RBC demonstrate increased adhesion to laminin
-increased expression & phosporylation of Lutheran
Can demonstrate Rh mosaicism
-Tn polyagg is form of mosaicism
Type 2 precursor Gal-GlcNAc
B1-4
Leukemia/carcinoma can decrease what antigens ABH & I
can reduce sialic acid, exposing T & Tn antigens
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
Usually makes anti-A1
Am subgroup Negative anti-A
Secretes A & H
Ael subgroup Negative anti-A
Secretes H only
Highest expression of H O>A2>B>A2B>A1>A1B> H deficient
Lowest expression of H
Lewis transferase & Sugar FUT-3
Fuctose alpha1-4
Type 1 H antigen transferase & sugar FUT-2
Fuctose alpha1-2
, SBB Exam Review
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
Band 3 macrocomplex of proteins including:
-Rh
-RhAG
-LW (ICAM-4)
-GPA
-GPB
-CD47
Ankryin mediates interaction between Band 3 and underlying rbc skeleton
attaches to spectrin through interaction with protein 4.2
Band 4.1 mediates interactions between GPC and underlying rbc skeleton
Adducin interacts with dimeric Band 3 (associated with GPC)
Important for maintaining membrane stability
Spectrin major component of underlying skeleton
consists of 2 subunits: alpha & beta
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
Allows rbc to regulate O2 transport during hypoxia
Embden-Meyerhoff Pathway Whole anaerobic glycolysis pathway taken by rbc
generates 90% of ATP needed for rbc
"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
, SBB Exam Review
Compensated Anemia physiologic reactions Increased:
-Cardiac output (heart rate, stroke volume)
- Blood flow (vasodilation, shunting)
- O2 extraction
- O2- Hgb affinity (shift right)
Dyserythropoiesis HEMPAS, sideroblastic anemia, megaloblastic anemia
Altered erythropoesis quantitatively and qualitatively
increased i
associated with Hypoglycosylation
Myeloproliferative Disease RBC demonstrate increased adhesion to laminin
-increased expression & phosporylation of Lutheran
Can demonstrate Rh mosaicism
-Tn polyagg is form of mosaicism
Type 2 precursor Gal-GlcNAc
B1-4
Leukemia/carcinoma can decrease what antigens ABH & I
can reduce sialic acid, exposing T & Tn antigens
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
Usually makes anti-A1
Am subgroup Negative anti-A
Secretes A & H
Ael subgroup Negative anti-A
Secretes H only
Highest expression of H O>A2>B>A2B>A1>A1B> H deficient
Lowest expression of H
Lewis transferase & Sugar FUT-3
Fuctose alpha1-4
Type 1 H antigen transferase & sugar FUT-2
Fuctose alpha1-2
, SBB Exam Review
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
