IM Hematology Flashcards
What is the physiologic cause of hemolytic anemia in G6PD deficiency?
Reduction in glutathione levels in red cells
What is the inheritance pattern of G6PD deficiency?
X-linked recessive
What ethnicities is G6PD deficiency most commonly seen in?
African and Mediterranean
Name some drugs that are likely to lead to acute hemolysis in patients with G6PD deficiency?
Oxidative drugs such as primaquine, aspirin, dapsone, quinidine, sulfonamides, and nitrofurantoin
What is seen on peripheral blood smear that is consistent with a hemolytic episode of G6PD deficiency?
Heinz bodies and bite cells
Is thalassemia a macrocytic or microcytic anemia?
Microcytic
What ethnicity is alpha thalassemia commonly found in?
Southeast Asian and Chinese
What ethnicity is beta thalassemia most commonly found in?
African and Mediterranean
How many types of alpha thalassemia are there? What are they?
4 types: Carrier - 1 alpha gene deletion Minor - two alpha gene deletions Hb H disease - 3 alpha gene deletions Hydrops fetalis - 4 alpha gene deletions
What chromosome is the alpha gene found on?
Chromosome 16
What is the inheritance pattern of sickle cell anemia?
Autosomal recessive
At what age does sickle cell anemia begin to present? Why?
Around 6 months, when fetal hemoglobin is replaced with HbS.
What is the most common cause of death due to sickle cell anemia?
Acute chest syndrome
What is the definitive diagnostic study for sickle cell anemia?
Electrophoresis
What is the only pharmacologic agent that has consistently demonstrated improvements in episodes of pain and survival rates for patients with sickle cell anemia?
Hydroxyurea