Hemolytic Anemia Flashcards
What is another name for hemolytic anemia?
Survival Defect
What is the RBC survival time in hemolytic anemia?
Less than 100 days
Hemolytic Anemia
- Classification (2)
- Grouped by cause of premature RBC destruction
Intravascular
Extravascular
Hemolytic Anemia
- Clinical Symptoms
- Jaundiced
- New onset of pallor and anemia
- Discoloration of the urine
- Gallstones
- Splenomegaly (preferential site of hemolysis)
Hemolytic Anemia
- Diagnostic Labs
- Haptoglobin decreased –> hemolysis
***Haptoglobin are dump trucks***
- LDH elevated in intra- and extravascular hemolysis
- Due to bursting open of cells - Coombs test positive
- Reticulocyte count elevated
- Indirect (i.e. unconjugated) bilirubin elevated
***Causes jaundice***
- Urine hemosiderin high –> intravascular hemolysis
G6PD Deficiency (causes hemolytic anemia
- Buzz Words
X-linked
Most Common enzymatic disorder
Fava Beans
Heinz Bodies
Bite Cells
Mnemonic: My X Girlfriend Bites Fava Beans with Heinz Ketchup
Bite Cells
Heinz Bodies
Pyruvate Kinase Deficiency
- Just know that it is in the realm of hemolytic anemia and is less common than G6PD deficiency
Sickle Cell Syndromes
- Mutation
Mutation in the B-globulin in which Valine is substituted in place of Glutamine
***Both B-globulin chains affected –> Sickle Cell Anemia (SCA)
What infections are patients with Sickle Cell Anemia more at risk for?
More at risk for infection by encapsulated organisms and Salmonella
(e.g. strep pneumoniae, meningococcal, H. influenzae, Hepatitis B, and Influenza vaccines)
Hereditary Spherocytosis and Eliptocytosis
- Mutation/Demographic
- Effects
- Complications
- Dx/Tx
Autosomal dominant in Northern Europeans
Loss of membrane flexibility and chronic hemolysis
Complications –> Cholelithiasis (d/t bili stones) splenomegaly
Dx: Osmotic Fragility Test
Tx: Folate Supplementation, Genetic Counseling, Splenectomy
Spherocytosis
Sphero/Eliptocytosis
Paroxysmal Nocturnal Hemoglobinuria (PNH)
- Mutation
2 Triad?
- Treatment
Defective PIG-A gene
Triad –> Hemolysis, Pancytopenia, Risk of Venous Thrombosis
Treatment: Hemolysis w/ corticosteroids, anticoagulants for thrombolytic events, Eculizumab - ab to C5 terminal complement - decreases hemolysis and need for transfusion
Autoimmune diseases will all usually respond to what treatment?
Treatment with steroids
Autoimmune Hemolytic Anemia
- Cause
- Symptoms
- Treatment
- Caused by IgG or IgM antibodies
- Clinically present with splenomegaly
- Treatment: Corticosteroids
If an attending ever asks you what could be the cause of something, always say what?
Drugs (drugs cause everything)
Drug-Associated Hemolysis
- Penicilins
- Quinine, methyldopa, certain cephalosporin antibiotics are known culprits
Functionally asplenic patients are at risk for what type of organisms?
Encapsulated (e.g. Strep pneumoniae)
and
Salmonella
What is the most common enzymatic defect in RBCs?
G6PD deficiency
Which 3 lab values and their respective changes would best reflect hemolytic anemia?
Indirect bilirubin elevated
LDH elevated
Reticulocyte count elevated
Heinz Bodies and Bite Cells are seen in which Hemolytic Anemia process?
G6PD Deficiency
Mnemonic: My X Girlfriend Bites Fava Beans with Heinz Ketchup
