HGB metabolism and Leukopoiesis

• Porphobilinogen deaminase in the presence of Porphobilinogen produces Hydroxymethylbilane
• Lead poisoning can inhibit Aminolevulinic dehydratase and Ferrochelatase
• The Gamma, beta, delta, epsilon globin are encoded by chromosome 11
• The Alpha and Zeta globin chains are encoded by Chromosome 16
• 2 gene-coded globins include Beta, Delta, Epsilon and Zeta
• 4 gene-coded globin include Alpha and Gamma
• Gower I composed of 2 Epsilon and 2 Zeta globin chains
• Gower II is composed of 2 Epsilon and 2 Alpha globin chain
• The Portland Globin chain composes of 2 Gamma and 2 Zeta
• The Fetal globin chain composes of 2 Alpha and 2 Gamma
• The Alpha 1 globin chain composes of 2 Alpha and 2 Beta
• The Alpha2 globin chain composes of 2 Alpha and 2 delta
• The normal HBA concentration in adult is 92-95%
• The normal HBA2 concentration in adult is 2-3%
• The normal HBF concentration in adult is 1-2%
• The affinity of oxygen is dependent on the partial pressure of oxygen on the site
• In every 1g of HB, there is 1.34 mL of oxygen
• P50 pertains to the amount of oxygen needed to saturate 50% of hemoglobin
• The normal P50 is around 27 mmHg
• The normal shape of an Oxygen Dissociation curve is sigmoidal
• The Bohr effect pertains to the shift in dissociation curve either left or right due to the changes in pH
• The Kleihauer-Betke test accounts for the ability of HGBF to resist acid denaturation as opposed to HGBA
• The Kleihauer-Betke test shows deep-red stained HGBF while HGBA are stained like pale-pink ghosts
• The normal values for Reticulocyte count is 0.5-2%
• The 2,3 DPG combines with the beta chains of the deoxyhemoglobin of rbc
• Deoxygenated hemoglobin exhibits the pulling apart of beta chains and allows establishment of salt bridges
• With oxygen uptake, loss of salt bridges happen and beta chains are pulled together expelling 2,3 DPG
• Alpha chains of the adult globin chains consists of 141 amino acids
• The gene for Erythropoietin is located at Chromosome 7
• The EPO is detectable at plasma with the values 20 mU/mL
• Normal bone marrow has a normal Reticulocyte production Index of 1
• Hemolytic anemias may show an RPI index of 3-7x higher than normal
• Deficiency in Hemoglobin components shows an RPI of less than 2
• In each beta chains of the globin, there are 146 amino acids
• The appearance of primary granules in neutrophil maturation can be seen in the promyelocyte
• The neutrophilic myelocyte stage shows an indented or D-shaped nucleus
• The neutrophilic myelocyte stage is the last stage of neutrophil maturation that is capable of mitosis
• The neutrophilic myelocyte stage is the first to produce secondary granules
• The neutrophilic myelocyte stage is the first stage in neutrophil maturation that allows differentiation of granulocytes
• The kidney-shaped nucleus can be seen in the Neutrophilic metamyelocyte stage in neutrophil maturation