Iron, Iron Disorders, Heme Disorders Flashcards

1
Q

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

A
  • transfusions

- prevent with chelation

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2
Q

What is ferritin?

A
  • large intracellular iron storage protein located in macrophages
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3
Q

What type of anemia is seen in iron deficiency anemia?

A
  • microcytic, hypochromic, hypo-proliferative anemia
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4
Q

Lead poisoning leads to what type of anemia?

A
  • microcytic anemia

* most often with “coarse basophilic stippling”

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5
Q

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

A
  • developmental delay
  • weight loss
  • abdominal pain
  • pica
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6
Q

Serum iron levels measure what?

A
  • Fe bound to transferrin
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7
Q

More hepcidin = ______ iron absorption

A

LESS

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8
Q

Iron studies in anemia of chronic disease usually reflects what?

A
  • sequestration of iron in RES
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9
Q

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

A
  • noncompliance
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10
Q

What site is the only regulatory point for iron absorption?

A

gut (duodenum)

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11
Q

How does lead poisoning lead to anemia?

A
  • anemia results from block in heme synthesis
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12
Q

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?

A
  • Acute intermittent porphyria (AIP)
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13
Q

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

A
  • Iron studies
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14
Q

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

A
  • Hepcidin
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15
Q

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

A
  • secondary to chronic hepatitis C or iron overload
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16
Q

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?

A
  • Iron deficiency anemia
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17
Q

What are the 2 etiologies of disorders of iron overload?

A
  • hereditary hemochromatosis

- secondary hemosiderosis

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18
Q

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?

A
  • porphyria cutanea tarda (PCT)
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19
Q

Total iron binding capacity (TIBC) measures what?

A
  • indirect measure of circulating transferrin
20
Q

How is hereditary hemochromatosis screened and diagnosed?

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

- confirm with genetic testing (HFE gene)

21
Q

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

A
  • GI blood loss
22
Q

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

A
  • colorectal cancer
23
Q

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

A
  • phlebotomy
24
Q

How is transferrin saturation calculated?

A

(Serum iron x 100)/TIBC

25
Q

What is the function of transferrin?

A
  • plasma transporter of iron
26
Q

What is the most common type of acute prophyrias?

A
  • acute intermittent porphyria (AIP)
27
Q

What type of genetic inheritance is seen in hereditary hemochromatosis?

A

autosomal recessive

28
Q

How is acute intermittent porphyria diagnosed and treated?

A
  • diagnosis = spot urine porphobilinogen

- treatment = IV glucose, hemin

29
Q

What is the most common cause of iron deficiency anemia?

A
  • Menorrhagia
30
Q

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

A
  • Ferritin = low
  • Serum iron = low
  • TIBC = elevated
  • transferrin sat = low
31
Q

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

A
  • Human homeostatic iron regulator (HFE) protein
32
Q

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

A
  • hepatocellular carcinoma
33
Q

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

A
  • hereditary hemochromatosis
34
Q

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

A
  • only in patients with severe symptoms, acute cardiac ischemia, or acute blood loss
35
Q

How is lead poisoning diagnosed and treated?

A
  • diagnosed = blood lead level

- treatment = chelation

36
Q

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

A
  • serum iron = low
  • Ferritin = high
  • TIBC = low
  • transferrin sat = low

pay attention to Ferritin + TIBC = opposite of iron deficiency anemia

37
Q

Ferritin can be falsely elevated in what condition?

A
  • inflammation

* it is an acute phase reactant

38
Q

Cheliosis and Koilonychia are findings that indicate severe _________.

A
  • iron deficiency anemia
39
Q

Serum ferritin levels measure what?

A
  • total body Fe stores (except for in inflammatory states)
40
Q

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

A
  • headache
  • abdominal pain
  • memory difficulties
  • HTN
41
Q

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

A
  • treatment = oral iron supplementation + Vitamin C

- side effect = constipation

42
Q

How is hereditary hemochromatosis treated?

A
  • first choice: phlebotomy

- iron chelating agents (deferoxamine)

43
Q

What is the cause of hereditary hemochromatosis?

A
  • mutations in the HFE gene resulting in reduced production of hepcidin
44
Q

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

A
  • anaphylaxis
45
Q

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

A
  • porphyria cutanea tarda (PCT)