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USMLE > Hematology > Flashcards

Hematology Flashcards

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USMLE® Step 1 flashcards
1
Q

Quickly think through the synthesis pathway of heme

A

GLycine + succinyl CoA - (ALAS)-> ALA -(ALAD)-> ProphoB -(ProphoB deaminase)->HMB -(UROS)->URO-(UROD)-> CoproP -(CoproP Oxidase)-> ProtoPgen -(ProtoP Oxidase)->ProtoP -(ferrochelatase)> Heme

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2
Q

Lead poisoning

  1. which enzymes affected?
  2. substrate and product of that enzymes?
  3. sx?
  4. treatment?
A
  1. ALAD, ferrochelatase
  2. ALAD turns ALA into ProphoB ; ferrochelatase turns ProtoP into heme
  3. LEAD: Lines (Bruton lines), Encephalopathy, Anemia (sideroblastic, basophilic stippling, Papperheimer body), Drops (wrist and foot drop) eDta and Dimercapterol is antidote
  4. EDTA, Dimercapterol
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3
Q

What disease affects ALAD? Features?

A

Lead poisoning, LEAD: Lines (Bruton lines), Encephalopathy, Anemia (sideroblastic, basophilic stippling, Papperheimer body), Drops (wrist and foot drop) eDta and Dimercapterol is antidote

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4
Q

What disease affects ALAD? Features?

A

Lead poisoning, LEAD: Lines (Bruton lines), Encephalopathy, Anemia (sideroblastic, basophilic stippling, Papperheimer body), Drops (wrist and foot drop) eDta and Dimercapterol is antidote

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5
Q

What disease affects UROD? Features? (3)

A
  • Prophyria Cutanea Tarda.
  • PCT (Phototosensitivity {blistering, hyperpigmented, chronic], associated with hep C)
  • raised hepatic transaminase
  • onycholysis
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6
Q

What disease affects UROS? Features?

A

Congenital erythropoietic prophyria

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7
Q

What disease affects UROS? Features?

A

Congenital erythropoietic prophyria

similar with PCT but children (congenital cause)

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8
Q

What disease affects ALAS? Features? (3)

A

X-linked sideroblastic anemia. X-linked recessive. Stippling basophilic RBC, Pappenheimer body)

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9
Q

What disease affects CoproP oxidase?

A

Hereditary coproprophyria

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10
Q

What disease affects ProtoPgen oxidase? Features? (3)

A

erythropoietic prophyria

photosensitivity, flaccid paralysis, stomach pain

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11
Q

What disease affects Ferrochelatase? Features?

A

Lead poisoning
LEAD: Lines (Bruton lines), Encephalopathy, Anemia (sideroblastic, basophilic stippling, Papperheimer body), Drops (wrist and foot drop)
eDta and Dimercapterol is antidote

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USMLE (19 decks)

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