APML Is Medical Emergency
11 Mar, 2021
Dr. Rajesh Bollam
can present w pancytopenia & associated sx (fatigue, SOB, bleed/bruise). Leukocytosis (like in this case) is a marker of high-risk disease
Dx: t(15,17) translocation (that results in fusion of PML-RAR gene). You don't need 20% blasts if that's present
cells are stuck in the promyelocyte stage
Prognosis: w/o tx, short term survival is very poor! Median survival is <1 mo. 
A major cause of this is hemorrhage! Up to 40% can have a fatal head bleed
That's why coagulopathy & APL = medical emergency! DIC 
bleeding)
Hyperfibrinolysis plasmin by tPA dependent mechanism 
fibrinolysis.
Do a neuro exam (head bleed in APL) Check Plt, D-dimer, fibrinogen, INR & have a low transfusion threshold: Fibrinogen >150, plt >30-50,INR <1.5
ATRA as soon as you *SUSPECT* APL (don't need confirmed dx)
ATRA promotes the terminal differentiation of malignant immature promyelocytes to mature neutrophils! 
Despite high early mortality, with treatment, remission rates can be >90% !!
Acute leukemia? Look for DIC/bleeding, TLS, febrile neutropenia, leukostasisPts. with APL have high short term mortality & high risk of bleeding. Make sure you check coags & do a neuro examStart ATRA if you suspect APL!
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