Anemia 3 Flashcards
What are the three classification schemes for hemolytic anemia?
Site of RBC destruction
Acquired vs congenital
MOA RBC desctruction
What are the signs/symptoms of hemolytic anemia, apart from the usual anemia symptoms?
Jaundice
Urine discoloration
Gallstones
Splenomegaly
What is the preferential site of RBC destruction in hemolytic anemia? What does this cause?
Spleen
Splenomegaly
What happens to haptoglobin in hemolysis? Why? How does this differ between intra and extravascular causes?
Decreased–bound to Hgb
No difference
What happens to LDH (lactate dehydrogenase) in hemolytic anemia? Why?How does this differ between intra and extravascular causes?
Elevated since this enzyme is found in RBCs, and is spilt out
No difference
What happens to the reticulocyte count with hemoyltic anemia?
Elevated (>100,000 suggest a hyperproliferative bone marrow)
What happens to the indirect bilirubin in hemolytic anemia?
Elevated
What happens to urine hemosiderin levels in hemolysis? How does this differ between intra and extravascular causes?
In intravascular ONLY, increased
Is LDH specific to hemolytic anemia?
Yes
What is the Coombs test result in hemolytic anemias?
Positive
What is the mode of inheritance for G6Pd deficiency?
X linked
What is the most common enzymatic deficiency that leads to hemolytic anemia?
G6PD deficiency
What are the findings on a PBS with G6PD deficiency?
Heinz bodies
Bite cells
What are the Heinz bodies seen in G6PD deficiencies?
chunks of denatured Hgb
What food should pts with G6PD deficiencies avoid at all costs?
Fava beans
What is the MOA of hemolytic anemia d/t pyruvate kinase deficiency?
Loss of structural proteins since there is a lack of ATP
What is the major difference between G6PD deficiency and pyruvate kinase deficiency?
Pyruvate kinase deficiency is not susceptible to increased oxidate stress
Are there periphearl blood smear abnormalities with pyruvate kinase deficiency?
No
What is the usual presentation of pyruvate kianse deficiency?
neonatal jaundice
True or false: sickle cell syndrome/disease usually has some degree of hemolytic anemia
True
Pts with sickle cell anemia are susceptible to what infections?
By encapsualted bacteria and salmonella
What causes the recurrent renal problems in pts with HbS?
recurrent infarcts
What are the symptoms of sickle cell trait?
Usually asymptomatic, but can have painless hematuria
What is Hgb SC disease?
Both HbS and HbC, causing rigid (but not sickled) Hgb
Why is a splenectomy used as a treatment for heriitary sphereocytosis?
Because spleen will capture any cells that are not correctly shaped
What are the two major complications of heriditary spherocytosis?
Splenomegaly
Cholelithiasis
both from dsetruction of RBCs
Is the spleen normally palpable?
No
What is the cause of paroxysmal nocturnal hmoglobinuria?
Defective PIG-A gene
What is the triad for paroxysmal nocturnal hemoglobinuria?
Hemolysis
Pancytopenia
Thromboses
How do you diagnose PNH?
Specific flwo cytometry assays for CD55 and CD59
What is the treatment for PNH?
corticosteroids
Anticoagulants
What two antibody types usually cause hemolytic anemia?
IgG or IgM
What is the test to check for autoimmune hemolytic anemia?
coombs test
What is the treatment for autoimmune hemolytic anemia?
Corticosteroids
Abs that react with RBC membrane proteins are of what type? What if they react to polysaccharides?
RBC proteins = IgG (warm)
Poly = IgM (cold)
What abx type is associated with hemolysis?
PCNs
Cephalosporins
