Hematology 1 PDF - FEU - AY 2024-2025

Hematology 1 AY 2024-2025 FAR EASTERN UNIVERSITY - DR. NICANOR REYES MEDICAL FOUNDATION (FEU-NRMF) Special Hematology Laboratory Tests Part 2 Sir Normel Adarve Hemoglobinopathies...

Hematology 1 AY 2024-2025 FAR EASTERN UNIVERSITY - DR. NICANOR REYES MEDICAL FOUNDATION (FEU-NRMF) Special Hematology Laboratory Tests Part 2 Sir Normel Adarve Hemoglobinopathies Unstable Hgb: 20 min Caused by amino acid substitution resulting to instability of the Hgb molecules which then A suspected presence Two (2) specimen are affects the normal shape of the RBC of Hgb is subjected to subjected for Hemoglobin problems are divided into two, an alcohol solution observation, one (1) quantitative and qualitative hemoglobin defect is refrigerated while THEY ARE ALSO KNOWN AS UNSTABLE HEMOGLOBIN Normal Hgb does not the other is incubated - Quantitative = more of a precipitate as quickly at 50℃ for 1 hour thalassemic/thalassemia type of defect as an unstable Hgb. *Quantitative disorders involve a problem or a However, The specimen precipitation test does subjected to defect in the number of the globin product (an not indicate what incubation for 1 hour imbalance in the number of globin chains)* specific unstable Hgb shows turbidity, - Qualitative = hemoglobinopathies, such as it is (could be Hgb: E, heavy precipitation sickle cell, wherein there’s a point of D, S travis, Charlem will occur compared mutation in the cell, resulting in a defect or Georgetown, PCH, to the other that was *Qualitative disorders involve a problem in the etc) refrigerated structure of the Hgb itself* Screening test for unstable Hgb *Only a screening *Tinitingnan ang ❖ Heat denaturation test test* optical density or ❖ Isopropanol precipitation test absorbance of light of both the refrigerated ❖ Heinz bodies test and the incubated* - visible Heinz bodies in the red blood cells under the microscope indicates a problem in the cells - Heinz bodies can be caused Sickle cell anemia by enzyme deficiencies, with One type of unstable Hgb one known deficiency being Beta globin chain is affected at the 6th position G6PD deficiency. of amino acid in the chain - The 6th position which is supposedly - Acetyl phenylhydrazine is Glutamic acid is replaced by Valine used to look for presence of Eight clinically significant forms Heinz bodies in red blood 1. SS cells - Hgb A1 is composed of two α and two β, if Laboratory test for unstable Hgb two β globin chain has mutation, from Isopropanol Heat Denaturation glutamic acid to valine, it is called Hgb SS Precipitation test test or Hemoglobin sickle cell anemia - AS - if only one β is affected, it is called Nonpolar solvents Unstable Hgb are heat sickle cell trait weakens the bonds of sensitive and will 2. SC Hgb molecule causing precipitate heavily 3. SD-Punjab it to precipitate when incubated at 4. SO-Arab 50OC for 1 hours 5. S-B thalassemia 6. SE 17% Alc + Hgb at Normal Hgb will show 7. S-heredity persistence fetal Hgb 37℃ small precipitate *Others Hgb that has S are called sickle cell diseases or disorders* Normal Hgb : 40 min 1 Aly.D  sickling occurs in the fetus. (it is required for the donor or the donated blood to be negative from Hgb S) When theres a point mutation, the electrical charges of the protein also changes, becoming unstable and precipitate hence the change of the red blood cell shape from its normal form to a sickle cell-like structure What triggers sickling of RBC ❖ Low oxygen tension - can be from high Test for sickle cell - screening altitude places Sodium metabisulfite (Sodium) Dithionite ❖ Dehydration - there’s a tendency of test or Sodium solubility test crystallization of Hgb molecule from the bisulfite test lack of fluid in the body ❖ Low blood pH or acidosis + - What happens when sickle cells are formed? Scenario 1:Lack of oxygen in the red blood cells turn into sickle RBCs in the spleen which are then eaten by the macrophages (removes abnormal rbcs). Kapag maraming tinanggal na (abnormal) RBCs si macrophages, pwedeng maging sanhi siya ng hemolytic anemia Vaso occlusive crisis Put sodium Principle: Scenario 2: Again, lack of oxygen in RBCs turn into metabisulfite in a Red cell + saponin → sickle cells. Those sickle cells become trapped blood smear - the Hgb + Sodium within the microvascular channels inside the sodium metabisulfite dithionite → Hgb S spleen, cutting off blood supply or blocking the cause oxidation and tactoids blood flow of the spleen or kidney or other organs precipitation in Hgb *REMEMBER - 3 disorders associated with sickle SS and AS, causing Saponin - to lyse the cell anemia: deformation on the red cell 1. Hgb SS - sickle cell anemia appearance of the RBC Sodium dithionite - 2. Hgb AS - sickle cell trait reducing agent, 3. Combination of other forms of variance of 2 types of causing precipitation Hgb with S - sickle cell disorders appearances produced or oxidation KAPAG after putting sodium may presence lang ng *Hemolytic disease of the newborn (HDN) — also metabisulfite: Hgb S (either SS or called erythroblastosis fetalis = Intrauterine ❖ Holly-leaf = AS), turning it into transfussion is done in the blood banking section most probably Hgb S tactoids to transfuse blood to a fetus inside the mother’s the Hgb AS (‘DI ❖ Look at the womb because there are wherein the blood of the LAGI, may turbidity of the mother and the fetus are not compatible, hence, tendency ‘din sample, turbid 2 Aly.D  kasi na ganito (cannot see the Cellulose acetate pH ang magpakita line of the 8.0 sa Hgb SS) background - alkaline ❖ Sickle = more that was used) environment probably it is = oxydized or - standard order of Hgb SS precipitated = Other Hemoglobins migration towards ❖ Only thing presence of present: the positive charge confirmed in Hgb S ❖ Hgb C - Hgb should be Hgb A, F, this screening A2, E, O-Arab S, C is that you ❖ Hgb S - Hgb D, - point of origin is have Hgb S G near the negative charge - Considered Question abnormal kapag mas mabilis Which of the following will give a false negative mag-migrate ang: sodium dithionite solubility? use 12 x 75mm test Bart’s Hgb, Hgb I, tube Hgb H kaysa sa a. Hgb of