Iron, Iron Disorders, Heme Disorders Flashcards
Secondary hemosiderosis is commonly caused by what? How can it be prevented?
- transfusions
- prevent with chelation
What is ferritin?
- large intracellular iron storage protein located in macrophages
What type of anemia is seen in iron deficiency anemia?
- microcytic, hypochromic, hypo-proliferative anemia
Lead poisoning leads to what type of anemia?
- microcytic anemia
* most often with “coarse basophilic stippling”
What are some clinical manifestations of lead poisoning in children? (4)
- developmental delay
- weight loss
- abdominal pain
- pica
Serum iron levels measure what?
- Fe bound to transferrin
More hepcidin = ______ iron absorption
LESS
Iron studies in anemia of chronic disease usually reflects what?
- sequestration of iron in RES
What is the main cause of failure to respond to treatment in iron deficiency anemia?
- noncompliance
What site is the only regulatory point for iron absorption?
gut (duodenum)
How does lead poisoning lead to anemia?
- anemia results from block in heme synthesis
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?
- Acute intermittent porphyria (AIP)
When administering EPO it is important to check what lab values first?
- Iron studies
This protein is a negative regulator of iron absorption and mobilization.
- Hepcidin
Porphyria cutanea tarda (PCT) is most often caused by what?
- secondary to chronic hepatitis C or iron overload
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?
- Iron deficiency anemia
What are the 2 etiologies of disorders of iron overload?
- hereditary hemochromatosis
- secondary hemosiderosis
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?
- porphyria cutanea tarda (PCT)
Total iron binding capacity (TIBC) measures what?
- indirect measure of circulating transferrin
How is hereditary hemochromatosis screened and diagnosed?
- screen with transferrin saturation (>60% M; >50% W)
- confirm with genetic testing (HFE gene)
Iron deficiency anemia in adult males should have you immediately concerned for what?
- GI blood loss
Iron deficiency anemia in OLDER males should have you immediately concerned for what?
- colorectal cancer
What is the treatment for Porphyria cutanea tarda (PCT)?
- phlebotomy
How is transferrin saturation calculated?
(Serum iron x 100)/TIBC
What is the function of transferrin?
- plasma transporter of iron
What is the most common type of acute prophyrias?
- acute intermittent porphyria (AIP)
What type of genetic inheritance is seen in hereditary hemochromatosis?
autosomal recessive
How is acute intermittent porphyria diagnosed and treated?
- diagnosis = spot urine porphobilinogen
- treatment = IV glucose, hemin
What is the most common cause of iron deficiency anemia?
- Menorrhagia
What findings would you expect on iron studies for iron deficiency anemia? (4 components)
- Ferritin = low
- Serum iron = low
- TIBC = elevated
- transferrin sat = low
This protein binds to transferrin receptor and mediates the action of hepcidin.
- Human homeostatic iron regulator (HFE) protein
Half of patients with hereditary hemochromatosis that present with multiorgan disease die of what?
- hepatocellular carcinoma
A “bronze tan” also known as “bronze diabetes” without associated sun exposure is a hallmark sign of what condition?
- hereditary hemochromatosis
When is pRBC transfusion indicated in the treatment of iron deficiency anemia?
- only in patients with severe symptoms, acute cardiac ischemia, or acute blood loss
How is lead poisoning diagnosed and treated?
- diagnosed = blood lead level
- treatment = chelation
What findings would you expect on iron studies for anemia of chronic disease? (4 components)
- serum iron = low
- Ferritin = high
- TIBC = low
- transferrin sat = low
pay attention to Ferritin + TIBC = opposite of iron deficiency anemia
Ferritin can be falsely elevated in what condition?
- inflammation
* it is an acute phase reactant
Cheliosis and Koilonychia are findings that indicate severe _________.
- iron deficiency anemia
Serum ferritin levels measure what?
- total body Fe stores (except for in inflammatory states)
What are some clinical manifestations of lead poisoning in adults? (4)
- headache
- abdominal pain
- memory difficulties
- HTN
What is the first line treatment for iron deficiency anemia? What is the primary side effect?
- treatment = oral iron supplementation + Vitamin C
- side effect = constipation
How is hereditary hemochromatosis treated?
- first choice: phlebotomy
- iron chelating agents (deferoxamine)
What is the cause of hereditary hemochromatosis?
- mutations in the HFE gene resulting in reduced production of hepcidin
What is the primary side effect with IV iron infusion for the treatment of iron deficiency anemia?
- anaphylaxis
What is the most common type of non-acute porphyrias?
- porphyria cutanea tarda (PCT)
