STEP 1: Heme
Terms
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Autoimmune Hemolytic Anemia:
Warm
Ig__
Seen in _____, _____, or with use of ____. -
G
SLE, CLL, a-methyldopa - Rh incompatibility between mother and child is a form of _____ _____ anemia.
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Warm Agglutinin
Autoimmune Hemolytic
Acute IgM -
1. Serum Iron low
Transferrin high
2. Serum Iron low
Transferrin low
3. Serum iron high
Transferrin low -
1. Iron def. anemia
2. Chronic dx anemia
3. Hemochromatosis - Porphyrias are caused by defects in ______ that lead to ________.
- Heme synthesis, accumulation of heme precursors
- causes inhibition of ferrrochelatase and ALA dehydroegenase
- Lead Poisoning
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Porphobilinogen deaminase
1. Condition
2. Accumulated substrate -
1. Acute intermittent porphyria
2. d-ALA, porphobilinogen - Pink / purple urine
- Acute intermittent porphyria
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Most common porphyria
Think Blisters -
Porphyria cutanea tarda
Defective enzyme: Uroporphyrinogen decarboxylase -
Tea colored urine
Blisters - Porpyria Cutana Tarda
- Treatment for Lead Poisoning
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Dimercaprol, EDTA
Succimer for kids
"Ed needed a dime to treat his lead poisoning"
"It sucks to be a kid with lead poisoning" - Anti G2b3a kills platelets
- ITP
- A lot of vWF multimers
- TTP
- No ADAMS 13 to _______.
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TTP
degrade vWF multimers - Tests EXtrinsic pathway / factors _____
- PT, 1,2,5,7,9
- Tests INtrinsic pathway, factors _____.
- PTT, all but 7 and 13
- PTT is up, easy bruising, bleeding in joints
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Hemophilias
A: no factor 8
B: no factor 9 -
PT and PTT up
Think these 2 scenerios:
____, _____
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Vitamin K deficiency
liver or kidney failure