Paroxysmal nocturnal hemoglobinuria Flashcards Preview

Hematology, oncology > Paroxysmal nocturnal hemoglobinuria > Flashcards

Flashcards in Paroxysmal nocturnal hemoglobinuria Deck (10)
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1
Q

What is the role for prophylactic AC?

A

no longer used since advent of eculizumab

2
Q

Disease association to know for boards with pNH

A

aplastic anemia

3
Q

Diagnosis of PNH

A

flow cytometry (looking for CD55 and CD59 deficiency)

4
Q

cell markers to know

A

CD55 + CD59

5
Q

VTE management in PNH patient

A

lovenox bridge to warfarin (confirm)

6
Q

First and second line for PNH

A

1) folate supplementation
2) IF large clone size –> eculiziumab (targets CD5, inhibiting complement) indefinitely (doesn’t correct underlying mutation)
3) second line = allo HCT

7
Q

Presentation of PNH

A
  • fatigue
  • jaundice
  • hemoglobinuria
  • abdominal pain/ED/pHTN (due to NO depletion)
8
Q

Hereditary or somatic?

A

acquired defect (somatic cell)

9
Q

Labs in PNH

A
  • nonspecific - variable RC size, variable retic count, elevated LDH,
  • this is why its a hard diagnosis to make
    ***can also see pancytopenic because it’s a somatic mutation in an HSCT
10
Q

What does initiation of therapy depend on in PNH?

A

Size of the clone

Decks in Hematology, oncology Class (218):