Flashcards in Patho-RBC and Bleeding Disorders Deck (23):
Erythropoeitin level in hemolytic anemias
Most common cause of IDA overall?
Intrinsic hemolysis is caused by
Intrinsic defects in rbc
How do u differentiate extravascular from intravascular hemolysis?
Extravascular hemolysis has splenomegaly
What is the expected morphology on peripheral blood smear of hemolytic anemias?
Hereditary spherocytosis is brought about by what mutations
In a patient with hereditary spherocytosis, infection with parvovirus b19 can cause?
Infection with EBV?
Parvo - aplastic crisis
EBV- hemolytic crisis
Small dark nuclear remnants present in RBCs of asplenic patients
G6pd is the rate limiting step in which biochemical pathway?
Pentose phosphate pathway
What is the function of G6PD
Reduces NADP to NADPH; provides reducing equivalents
Morphology G6PD deficiency
What are heinz bodies?
Membrane bound precipitates on denatured globin chains
Mutation in sickle cell anemia
Point mutation in 6th codon of beta globin
Replacement of glutamate with VALINE
Dehydrated RBCs with bulls eye apoearance seen in sickle cell disease
Why are infants with sickle cell disease asymptomatic until 2-4 mos of age?
Due to high fetal hemoglobin F
Crises in Sickle cell anemia
Most common manifestation of vasoocclusive crisis in sickle cell anemia?
Hand foot syndrome
Inflammation of finger and toe
Massive entrapment of sickle cells leads to rapid splenic enlargement, hypovolemia, and shock
Renal manifestation of sickle cell anemia
Inability to concentrate urine
Signs of extramedullary hematopoieisis in sickle cell
Crew cut skull
What is the best initial diagnostic test for sickle cell anemia?
Not used in newborns
What is the drug of choice for sickle cell anemia and what is its mechanism of action?
Hydroxyurea (increases HbF)