APML – 12/5/17

  • Makes 5-20% of AML cases and accounts for 600-800 cases per year in the US
  • Higher incidence in people from Mexico, Central America, South America, Italy, and Spain
  • Uncommon < 10 years old and > 60 years old
  • Considered a medical emergency due to a high rate of mortality from hemorrhage

Labs will show:

  • Pancyctopenia (one of the few leukemias that classically can present with a low WBC)
  • Peripheral smear with promyelocytes and high nucleus to cytoplasm ratio with granules
  • Coagulopathy (frequently with DIC)
  • FISH with t(15:17) translocation

Treatment

  • ATRA – start immediately even before diagnosis is confirmed given high rate of mortality without treatment

Consequences of ATRA

  • ATRA differentiation syndrome – presents with SOB and volume overload – can occur at week 1 of treatment or up to 3-4 weeks after – treatment is dexamethasone and patient can be continued on ATRA
  • Pseduotumor cerebri – look for in patients with high ICP – pathophysiology due to ATRA causing a hypervitaminosis A type syndrome – do LP to check ICP

Platelet transfusion goals

  • > 100 for NSG procedure
  • > 50 for bedside procedure or surgery
  • >10 for anyone without bleeding to prevent spontaneous bleeding

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s