Sideroblastic anemia and Hemachromatosis (Exam 2)

Question 1

What are the 4 causes of sideropenic anemia?

Answer 1

low iron intake | poor absorption | increased need for iron | excessive iron loss

Question 2

What is sideroachrestic anemia?

Answer 2

have iron but can’t use it

Question 3

What are the 2 ways to have sideroblastic anemia?

Answer 3

genetics | acquired

Question 4

What are 4 ways one can acquire sideroblastic anemia?

Answer 4

rheumatoid arthritis | drugs | cancer | alcohol

Question 5

What is the mechanism of disease of sideroblastic anemia?

Answer 5

dysfunctional heme synthesis | has iron available but cannot incorporated it into Hb due to defect in protoporphyrin synthesis

Question 6

What are the findings of sideroblastic anemia?

Answer 6

serum iron (high) | ferritin levels (high) | transferrin saturation (high)

Question 7

What is a defining characteristic of sideroblastic anemia?

Answer 7

ringed sideroblasts around nucleus

Question 8

What are the findings of sideroblastic anemia due to?

Answer 8

iron can’t be used since there isn’t enough functioning protoporphyrin that will bind to available iron = left over iron

Question 9

What are ringed sideroblasts?

Answer 9

produced by BM in lieu of normal RBCs in sideroblastic anemia because no protoporphyria &raquo_space;> accumulation of iron granules in a ring form

Question 10

What is the size of the RBCs of sideroblastic anemia?

Answer 10

small due to not enough heme = reduced number of hemoglobinization

Question 11

What is the treatment for sideroblastic anemia?

Answer 11

blood transfusion | BM transplantation for severe cases

Question 12

What other disease does sideroblastic anemia can develop into? How?

Answer 12

too much iron in sideroblastic anemia can lead to hemochromatosis

Question 13

What is the mechanism of disease of hemochromatosis?

Answer 13

excessive iron intake &raquo_space;> iron stored as ferritin = deposited into cells and organs (bc its insoluble) = abnormal functioning of organs

Question 14

What is an early presentation of hemochromatosis and why?

Answer 14

diabetes | due to ferritin iron deposited in the pancreas = destroys cells that make insulin = diabetes

Question 15

What are the 3 types of hemochromatosis? Which is the most severe?

Answer 15

primary | hereditary (severe) | idiopathic

Question 16

What is idiopathic?

Answer 16

cause is unknown

Question 17

Which individuals are commonly affected with hemochromatosis?

Answer 17

males

Question 18

What age group does 70% of the clinical presentation occur in?

Answer 18

40-50 y/o

Question 19

What are the findings of hemochromatosis?

Answer 19

transferrin saturation (high) | UIBC and TIBC (low)

Question 20

What is the % of people with hemochromatosis develop onset diabetes? What is this due to?

Answer 20

65% | due to iron deposit in pancreas

Question 21

What are the 4 symptoms of hemochromatosis?

Answer 21

skin (and organ) bronze pigmentation | weight loss | hair loss | abdominal pain

Question 22

What is the most effective treatment for hemochromatosis?

Answer 22

remove blood on regular basis