Anemia 3

Question 0

Microcytic Anemias

Answer 0

Iron Deficiency Anemia
Anemia of Chronic Disease
Sideroblastic Anemia
Thalassemia

Question 1

Microcytic anemias are due to reduction in Hemoglobin. The Heme part of Hb is made of what?

Answer 1

Iron and Protoporforin

Question 2

What cell is responsible for the absorption of iron in the duodenum?

Answer 2

Enterocytes

using Ferroportin which is the transporter

Question 3

Iron in storage is in the form of _____

Iron in blood is bound to _____

Answer 3

1. ferritin (in bone marrow macrophages)
2. bound to Transferrin (b/c iron will form free radicals if not bound)

TIBC measures how much Tf in blood

Question 4

Gastrectomy causes iron deficiency anemia. Why?

Answer 4

Gastrectomy = losing part of stomach, therefore less acid secretion.
Iron stays in the Fe2+ state in acidic environment, and that is the form that is more readily absorbed. Fe3+ is less so. (Fe2+ goes in 2 the body)

Question 5

What is the normal percent saturation of Transferrin?

Answer 5

2/6 or 33% This number goes down when serum iron is depleted

Question 6

What is koilonychia?
What is pica?
Seen in?

Answer 6

Koilonychia: spoon shaped nails
Pica: chewing on things that are not food

Seen in iron deficiency anemia

Question 7

Free Erythrocyte Protoporforin

Increases in iron deficiency anemia. Why?

Answer 7

Hemoglobin is Heme + Protoporforin

so if reduction in Heme, then there will be free FEP

Question 8

Plummer Vinson Syndrome

What is it? Presentation?

Answer 8

Iron Deficiency Anemia with esophageal web (an obstruction) and atrophic glossitis (swelling of tongue)

Presents: anemia, dysphagia, beefy red tongue

Question 9

Hepcidin is a acute phase protein found in inflammation. What Anemia is it associated with? What is its MOA?

Answer 9

Anemia of Chronic Disease
Hepcidin sequesters iron in storage sites, and limits transfer from macrophages to erythroid precursor cells.

This essentially hides iron away from bacteria, only in chronic disease there is no bacteria.

Question 10

Sideroblastic anemia due to low ____

Answer 10

protoporphyrin

Question 11

How are ring sideroblasts formed?

Answer 11

Iron is absorbed into erythroid precursor cells, where they are bound to protoporphyrin in mitochondira. If protoporphyrin is deficient, iron remains trapped in mitochondira. Forming a ring around the nucleus.

Question 12

Congenital sideroblastic anemia is due to defect in what enzyme?

Answer 12

ALAS, the rate limiting enzyme involved in the coversion of Succinyl CoA to ALA, which is then converted into protoporphyrin, which is combined with iron to form heme.

Question 13

Acquired Sideroblastic Anemia causes

Answer 13

Alcoholism (mitochondrial poison)
Lead poisoning (inhibits ALAD and ferrochelitase)
Vit B6 deficiency (ALAS needs B6 as a cofactor)

Question 14

Thalassemia is decrease in production of

Answer 14

Globin chains

Microcytic Anemia

Question 15

Three normal types of hemoglobin

Answer 15

HbF (a2y2)
HbA (a2b2)
HbA2 (a2d2)

There are four copies of the alpha gene

Question 16

is Cis or Trans alpha gene deletion worse in Thalassemia?

Answer 16

Cis, because four genes total. Two from each parent. Cis means two deletions on one chromosome (16), and trans means two deletions, one on each chromosome. A Cis deletion that is passed down to offspring has a higher chance of causing severe thalassemia of the other parent happens to have another deletion, causing 3 deletions. 4 deletions causes death in utero.

Question 17

3 gene deletion forms HbH, what does this look like?

Answer 17

Tetramer of Beta globins B2B2

Question 18

4 gene deletion in Thalassemia causes Hb Barts, what does this look like?

Answer 18

Tetramer of gammas, d2d2

Causes hydrops fetalis

Question 19

beta Thalassemia is due to gene deletion or mutation?

Answer 19

Mutation. Two beta genes exist on chromosome 11

Question 20

Beta thalassemia minor increase in which Hb?

Answer 20

HbA2

Question 21

Beta thalassemia minor doesn’t present while baby is in womb because…

Answer 21

Baby has HbF (no beta globin), but as it develops, there will be demand for HbA