Iron, Iron Disorders, Heme Disorders

Question 1

Secondary hemosiderosis is commonly caused by what? How can it be prevented?

Answer 1

• transfusions

- prevent with chelation

Question 2

What is ferritin?

Answer 2

• large intracellular iron storage protein located in macrophages

Question 3

What type of anemia is seen in iron deficiency anemia?

Answer 3

• microcytic, hypochromic, hypo-proliferative anemia

Question 4

Lead poisoning leads to what type of anemia?

Answer 4

• microcytic anemia

* most often with “coarse basophilic stippling”

Question 5

What are some clinical manifestations of lead poisoning in children? (4)

Answer 5

• developmental delay
• weight loss
• abdominal pain
• pica

Question 6

Serum iron levels measure what?

Answer 6

• Fe bound to transferrin

Question 7

More hepcidin = ______ iron absorption

Answer 7

LESS

Question 8

Iron studies in anemia of chronic disease usually reflects what?

Answer 8

• sequestration of iron in RES

Question 9

What is the main cause of failure to respond to treatment in iron deficiency anemia?

Answer 9

• noncompliance

Question 10

What site is the only regulatory point for iron absorption?

Answer 10

gut (duodenum)

Question 11

How does lead poisoning lead to anemia?

Answer 11

• anemia results from block in heme synthesis

Question 12

A patient presents with red urine and severe abdominal pain out of proportion to other findings on physical exam. What diagnosis are you concerned about?

Answer 12

• Acute intermittent porphyria (AIP)

Question 13

When administering EPO it is important to check what lab values first?

Answer 13

• Iron studies

Question 14

This protein is a negative regulator of iron absorption and mobilization.

Answer 14

• Hepcidin

Question 15

Porphyria cutanea tarda (PCT) is most often caused by what?

Answer 15

• secondary to chronic hepatitis C or iron overload

Question 16

A patient presents with fatigue and low energy. He states he is constantly chewing on ice. On physical exam you notice skin pallor. What diagnosis are you concerned about?

Answer 16

• Iron deficiency anemia

Question 17

What are the 2 etiologies of disorders of iron overload?

Answer 17

• hereditary hemochromatosis

- secondary hemosiderosis

Question 18

A patient presents complaining of a painful blistering photosensitive skin rash. His skin feels fragile, and you notice excessive hair growth. What diagnosis are you concerned about?

Answer 18

• porphyria cutanea tarda (PCT)

Question 19

Total iron binding capacity (TIBC) measures what?

Answer 19

• indirect measure of circulating transferrin

Question 20

How is hereditary hemochromatosis screened and diagnosed?

Answer 20

• screen with transferrin saturation (>60% M; >50% W)

- confirm with genetic testing (HFE gene)

Question 21

Iron deficiency anemia in adult males should have you immediately concerned for what?

Answer 21

• GI blood loss

Question 22

Iron deficiency anemia in OLDER males should have you immediately concerned for what?

Answer 22

• colorectal cancer

Question 23

What is the treatment for Porphyria cutanea tarda (PCT)?

Answer 23

• phlebotomy

Question 24

How is transferrin saturation calculated?

Answer 24

(Serum iron x 100)/TIBC

Question 25

What is the function of transferrin?

Answer 25

- plasma transporter of iron

Question 26

What is the most common type of acute prophyrias?

Answer 26

- acute intermittent porphyria (AIP)

Question 27

What type of genetic inheritance is seen in hereditary hemochromatosis?

Answer 27

autosomal recessive

Question 28

How is acute intermittent porphyria diagnosed and treated?

Answer 28

- diagnosis = spot urine porphobilinogen | - treatment = IV glucose, hemin

Question 29

What is the most common cause of iron deficiency anemia?

Answer 29

- Menorrhagia

Question 30

What findings would you expect on iron studies for iron deficiency anemia? (4 components)

Answer 30

- Ferritin = low - Serum iron = low - TIBC = elevated - transferrin sat = low

Question 31

This protein binds to transferrin receptor and mediates the action of hepcidin.

Answer 31

- Human homeostatic iron regulator (HFE) protein

Question 32

Half of patients with hereditary hemochromatosis that present with multiorgan disease die of what?

Answer 32

- hepatocellular carcinoma

Question 33

A "bronze tan" also known as "bronze diabetes" without associated sun exposure is a hallmark sign of what condition?

Answer 33

- hereditary hemochromatosis

Question 34

When is pRBC transfusion indicated in the treatment of iron deficiency anemia?

Answer 34

- only in patients with severe symptoms, acute cardiac ischemia, or acute blood loss

Question 35

How is lead poisoning diagnosed and treated?

Answer 35

- diagnosed = blood lead level | - treatment = chelation

Question 36

What findings would you expect on iron studies for anemia of chronic disease? (4 components)

Answer 36

- serum iron = low - Ferritin = high - TIBC = low - transferrin sat = low *pay attention to Ferritin + TIBC = opposite of iron deficiency anemia*

Question 37

Ferritin can be falsely elevated in what condition?

Answer 37

- inflammation | * it is an acute phase reactant

Question 38

Cheliosis and Koilonychia are findings that indicate severe _________.

Answer 38

- iron deficiency anemia

Question 39

Serum ferritin levels measure what?

Answer 39

- total body Fe stores (except for in inflammatory states)

Question 40

What are some clinical manifestations of lead poisoning in adults? (4)

Answer 40

- headache - abdominal pain - memory difficulties - HTN

Question 41

What is the first line treatment for iron deficiency anemia? What is the primary side effect?

Answer 41

- treatment = oral iron supplementation + Vitamin C | - side effect = constipation

Question 42

How is hereditary hemochromatosis treated?

Answer 42

- first choice: phlebotomy | - iron chelating agents (deferoxamine)

Question 43

What is the cause of hereditary hemochromatosis?

Answer 43

- mutations in the HFE gene resulting in reduced production of hepcidin

Question 44

What is the primary side effect with IV iron infusion for the treatment of iron deficiency anemia?

Answer 44

- anaphylaxis

Question 45

What is the most common type of non-acute porphyrias?

Answer 45

- porphyria cutanea tarda (PCT)