Hematologic Flashcards
What are symptoms of iron deficiency?
Pica, pagophagia
Koilonychia
Angular chelitis
Plummer-vinson (dysphagia + esophageal webs + atrophic gastritis + Fe deficiency)
How do you diagnose iron deficiency?
Fe < 150
CBC (microcytic, hypochromic):
Low MCV, MCHC, Fe, ferritin, & transferrin
Elevated RDW & TIBC
What are causes of iron deficiency?
MC = Blood loss (menstruation, occult - colon CA, hookworms)
Diet deficiency: pregnancy, breastfed infants
What are features of thalassemia?
Decreased production of globin chains
Microcytic w/ normal or increased Fe
What are the 4 types of alpha thalassemia? How many alleles are affected in each type?
- Silent carrier (1/4 alleles): asx
- Minor (2/4): mild microcytic anemia
- Intermedia aka Hgb H (3/4): chronic anemia, pallor, hepatosplenomegaly,frontal & maxilla bony overgrowth, gallstones, iron overload. Presents similar to beta-thal*
- Hydrops fetalis (4/4): stillbirth, Hgb Barts* (gamma tetramers)
How do you dx alpha thalassemia?
CBC:
Decreased MCV
Normal or increased RBC count
Normal or increased Fe
Peripheral smear:
Target cells*, teardrop cells, basophilic stippling
Heinz bodies in Hgb H
Hgb electrophoresis
How do you treat mild alpha thalassemia?
No tx needed
How do you treat moderate alpha thalassemia?
Folate (if retic count is high)
Avoid oxidative stress (s/a sulfa)
Avoid iron supplementation
How do you treat severe alpha thalassemia?
Weekly blood transfusions, vit C, folate
Iron chelating agents: IV Deferoxamine, PO deferasirox
Splenectomy (if refractory)
Allogeneic bone marrow transplant = definitive
Who is alpha thalassemia MC in? Who is beta thalassemia MC in?
Alpha: SE Asian
Beta: Mediterranean*, Africans
What are the types of beta thalassemia? How many alleles are affected in each type?
- Trait aka minor (1/2 alleles): asx or mild-mod anemia
- Intermedia: anemia, hepatosplenomegaly, bony disease
- Major aka Cooley’s anemia (2/2 alleles): become symptomatic at 6 mos, frontal bossing & maxillary overgrowth, hepatosplenomegaly, severe hemolytic anemia, gallstones, iron overload
How do you diagnose beta thalassemia?
CBC:
Decreased MCV
Normal or increased RBC count
Normal or increased Fe
Peripheral smear: target cells*, teardrop cells, basophilic stippling, nucleated RBCs
Hgb electrophoresis: HgbF increased, HgbA2 increased, HgbA decreased
Skull bossing on xray (“hair on end”)
How do you treat beta thal trait (minor)?
Offer genetic counseling
How do you treat beta thal major?
Periodic blood transfusions, vit C, folate
Avoid excess Fe
Iron chelating agents: IV deferoxamine, PO deferasirox
Splenectomy if refractory
Allogenic bone transplant = definitive
What vitamin increases the absorption of Fe absorption?
Vit C!