14. Hematology Flashcards
Intrinsic vs. extrinsic hemolytic anemia?
Intrinsic - something wrong with the RBC that causes it to hemolyze vs. extrinsic - RBC is totally fine and something bad happens to it
Lack of central pallor?
Hereditary spherocytosis
What’s the inheritance of hereditary spherocytosis?
Autosomal dominant
Why do patients with spherocytosis have splenomegaly?
Spleen is what clears these cells
What is the diagnostic test for spherocytosis?
Osmotic fragility
What is the treatment for spherocytosis?
Splenectomy
At what percentage of HbS do you get sickling?
60% - can get spontaneous sickling
Why do we get pain crises in sickle cell?
Vasoocclusion from sickle cells –> ischemia –> pain
What happens to the spleen eventually in sickle cell?
Autosplenectomy
What does Howell Jolly bodies tell you about the spleen?
Spleen isn’t working; splenic macrophages should remove Howell Jolly
When can a kid get their first sickling crisis (dactylitis)?
6 - 9 months of age because HbF inhibits sickling (protective for newborns)
What is the cause of osteomyelitis in sickle cell patients?
Salmonella (encapsulated)
What drug can we give for sickle cell?
Hydroxyurea - promotes HbF synthesis
What are the genetics of G6PD deficiency?
X linked recessive
Why do we need G6PD?
To regenerate glutathione so we can neutralize peroxide
