Flashcards in EXAM #1: HEMOLYTIC ANEMIA Deck (26):
What is the Hb definition of anemia in males? Females?
Males= less than 13.5 g/dL
Females= less than 12.5 g/dL
What is the base definition of "hemolytic" anemia?
Destruction of RBCs that exceeds the bone marrows ability to produce RBCs
What are the two major subdivisions of hemolytic anemia?
Clinically, what do most patients with hemolytic anemia present with?
Unconjugated/ indirect bilirubin
What are the clinical signs that are specific to hemolytic anemia?
- New onset pallor and dyspnea
- Discoloration of urine
What are the labs that are diagnostic to hemolytic anemia?
1) Decreased haptoglobin
2) Lactate dehydrogenase (LDH) elevated
3) Elevated retic.
4) Elevated indirect/unconjugated bilirubin
5) High urine hemosiderin
****"Haptoglobin are dump-trucks"****
Why is Haptoglobin decreased in hemolytic anemia?
- Haptoglobin binds free Hb in the blood and transports it
- Haptoglobin is LOW b/c destruction of RBCs leaves free Hb in the serum
Why is LDH elevated in hemolytic anemia?
- LDH is an enzyme in RBCs
- Rupture of cells releases LDH into the serum
What is a positive Coombs test indicative of?
Antibody or complement mediated hemolysis
What does an elevated corrected retic. count mean?
RBC production is working
List the labs that need to be drawn for a suspected hemolytic anemia.
What is the most common disorder that causes hemolytic anemia?
List the characteristics of G6PD.
- African and Mediterranean
- Unable to regenerate reduced glutathioine b/c of a lack of NADPH from HMP shunt
What are the abnormal cell types that are specific to G6PD?
List the characteristics of Pyruvate Kinase Deficiency.
- Less common than G6PD but causes MORE SEVERE hemolytic anemia
- Often presents in newborns w/ neonatal jaundice
*****Hemolytic crisis without exposure to oxidative agents*****
What is the mutation that causes Sickle Cell Syndrome?
Mutation of Beta-globin in which valine replaces glutamine
What is the difference between Sickle Cell Trait and Disease?
Trait= 1x Beta-globin affected
What type of Hb is seen in Sickle Cell Anemia?
What are the clinical manifestations of Sickle Cell Anemia?
1) Tissue infarction
2) Recurrent renal infarcts leading to isosthenuria
3) Recurrent splenic infarcts leading to asplenia
What infections are patients with Sickle Cell Disease at risk for?
Asplenia= susceptibility to encapsulated microorganisms:
1) S. pneumonia
3) H. influenzae
4) Hep B
What is Hereditary Spherocytosis?
Autosomal dominant disease effecting the RBC cytoskeletal proteins, resulting in spherical RBCs that undergo extravascular hemolysis
What are the complications of Hereditary Spherocytosis?
How is Hereditary Spherocytosis diagnosed?
Osmotic Fragility Test
How is Hereditary Spherocytosis treated?
- Folate supplementation
What is the typical treatment for autoimmune hemolytic anemia?