14 prognosis

Question 1

hereditary spherocytosis (HS)

Answer 1

-increased risk sepsis

Question 2

sickle cell anemia

Answer 2

• in kids: autosplenectomy

- 90% survive to age 20, 50% survive to 50+.

Question 3

B thalassemai major

Answer 3

• if chronic transfusinos, til 20s

- if no tx, early death d/t anemia

Question 4

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Answer 4

• Leading COD: Venous thrombosis (40%) of the hepatic, portal or cerebral vv.’s
• 5-10% develop acute myeloid leukemia (AML) or myelodysplastic syndrome.

Question 5

pernicious anemia

Answer 5

-Atrophy and metaplasia of gastric mucosa.
-Increased risk of gastric carcinoma, with no change in risk after Tx.
-Risk of atherosclerosis and thrombosis.
-Increased homocysteine.
-Neuro disease is due to accumulation of methylmalonic acid in the spinal cord.
o Parenteral or high dose B12 cures the anemia and will halt or reverse the peripheral neurologic disease.