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Hemolytic Anemia

Primary product of hemolysis Unconjugated Bilirubin
Where does unconjugated bilirubin get conjugated? The Liver
What is haptoglobin? A protein that binds to free Hb released from erythrocytes. Decrease in Haptoglobin indicates hemolytic anemia.
What measures indicate hemolysis? Increase in unconjugated bilirubin Decrease in haptoglobin Presence of Hemosiderin in urine Increase in blood LDH levels
What glycolysis products are used in RBC? 1) ATP 2) NADH - prevents methemoglobin build up 3) Reduced Glutathione - prevents ROS 4) 2,3 DPG - helps to release O2
Where is a common place for problems in patients with RBC disorders? Why? Spleen - they have splenic cords that RBC have to go through in circulation, requiring temporary membrane deformation. Any problem with temporary deforming the membrane will get stuck or damaged.
What are the classifications of Hemolytic Anemia? Intra-corpuscular (mostly inherited) vs. Extra-corpuscular (mostly aquired) Intravascular vs extravascular
What are some intracorpuscular diseases? Hereditary Spherocytosis, PNH, G6PD, Sickle Cell Anemia, Thalassemia,
What does presence of spherocytes in blood smear indicate? Hereditary Spherocytosis, Cold IgG
What protein is missing in people with Paroxysmal Nocturnal Hemoglobinuria (PNH)? They are missing GPI proteins (ex: CD55, CD59), which protects the RBC from destruction
What do you see in patients with G6PD patients and why? Heinz Bodies - This is because G6P Dehydrogenase that protects RBC from oxidative stress is missing -> Hb is oxidized and precipitate -> Precipitate is Heinz Bodies
Describe hereditary pattern of G6PD X-linked & More common in African and Mediterranean (African variant is milder than Mediterranean)
What are some extracorpuscular diseases? Warm IgG, Cold IgM = Cold Agglutinin
What test do you use to test for IgG or IgM associated hemolytic anemia? Direct Antiglobulin Test (DAT)/Direct Coomb's Test
What causes Hereditary Spherocytosis? Defect in ankryin, spectrin, or other membrane holding proteins -> membranes start losing membrane volume and becomes spherocytes = less chance for survival
Describe the Cold IgM anemia. At low temperatures, IgM binds to RBC and leads to complement cascade -> MAC complex leads to intravascular hemolysis Cold IgM is associated with Mycoplasma pneumoniae and infectious mononuclei (Epstein Barr Virus) = Mono
List diseases under the category of Thrombotic Microangiopathy Anemia TTP, Hemolytic Uremic Syndrome, DIC
What three symptoms are show in TMA? Hemolytic anemia + Thrombocytopenia + Schistocytes = Thrombotic Microangiopathy
Created by: soobyung92