• 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