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Hemolytic Anemia
Question | Answer |
---|---|
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 |