Hemolytic Anemia
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| Primary product of hemolysis | Unconjugated Bilirubin
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| Where does unconjugated bilirubin get conjugated? | The Liver
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| What is haptoglobin? | A protein that binds to free Hb released from erythrocytes.
Decrease in Haptoglobin indicates hemolytic anemia.
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| What measures indicate hemolysis? | Increase in unconjugated bilirubin
Decrease in haptoglobin
Presence of Hemosiderin in urine
Increase in blood LDH levels
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| 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
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| 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.
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| What are the classifications of Hemolytic Anemia? | Intra-corpuscular (mostly inherited) vs. Extra-corpuscular (mostly aquired)
Intravascular vs extravascular
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| What are some intracorpuscular diseases? | Hereditary Spherocytosis, PNH, G6PD, Sickle Cell Anemia, Thalassemia,
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| What does presence of spherocytes in blood smear indicate? | Hereditary Spherocytosis, Cold IgG
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| 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
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| 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
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| Describe hereditary pattern of G6PD | X-linked & More common in African and Mediterranean (African variant is milder than Mediterranean)
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| What are some extracorpuscular diseases? | Warm IgG, Cold IgM = Cold Agglutinin
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| What test do you use to test for IgG or IgM associated hemolytic anemia? | Direct Antiglobulin Test (DAT)/Direct Coomb's Test
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| 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
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| 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
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| List diseases under the category of Thrombotic Microangiopathy Anemia | TTP, Hemolytic Uremic Syndrome, DIC
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| What three symptoms are show in TMA? | Hemolytic anemia + Thrombocytopenia + Schistocytes = Thrombotic Microangiopathy
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