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|Primary product of hemolysis
|Where does unconjugated bilirubin get conjugated?
|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