Early Recognition

  • APL is a unique form of acute leukemia, and is different from other leukemias. Manage the treatment of your APL patient accordingly.
  • One of the most significant features of APL is the severe coagulopathy at onset often leading to severe life-threatening hemorrhagic or more rarely thrombotic events, which usually occurs within the first hours or days after APL diagnosis or even before a definitive APL diagnosis has been made and before the start of therapy.
  • If you suspect APL, initiate treatment quickly at the first suspect to prevent this life-threatening complication.
  • Although APL is the most curable acute leukemia in adults, early recognition and prompt institution of aggressive supportive therapy and specific therapeutic agents (all-trans retinoic acid and/or arsenic trioxide) can mitigate the associated severe hemorrhagic risk.

Pre-Diagnosis

  • Have a high suspicion of APL for any diagnosed leukemia patient exhibiting infiltration of bone marrow or peripheral blood by leukemic promyelocytes, evidence of laboratory coagulopathy and/or clinical signs of bleeding or thrombosis.

Diagnosis

  • Include diagnostic testing for the PML/retinoic acid alpha receptor (RARA) fusion gene via polymerase chain reaction (PCR), fluorescent in situ hybridization (FISH), or conventional cytogenetics of peripheral blood or bone marrow.

Essential Care

  • Recognize that APL is a medical emergency and rapidly initiate treatment with all-trans retinoic acid (ATRA).
  • Be able to suspect APL based on morphology, clinical manifestations and laboratory data.
  • Do not wait for genetic confirmation of the disease but immediately initiate treatment with all-trans retinoic acid (ATRA) and intensive blood product support when APL is suspected.
  • Maintain a platelet count of at least 30-50 000/μL and fibrinogen of 150 mg/dL.
  • Educate “intensive care” medical and nursing staff and other medical colleagues about APL specific complications and their management, i.e. bleeding, thrombosis, multi-organ complications, APL differentiation syndrome.
  • Frequently monitor laboratory test results for coagulopathy, blood counts and tumor lysis and alert nurses to monitor respiratory status closely and vital signs frequently.
  • Avoid any unnecessary procedures that are at risk of bleeding or thrombosis like central line placement (peripherally inserted central catheter [PICC] line is acceptable) and lumbar puncture in the immediate setting.
  • Ensure ATRA is available on formulary at all times.
  • Ensure arsenic trioxide (ATO) is available on formulary at all times.
  • Ensure that the pharmacist will dispense the medication immediately or that you have it on ward.