Acute Promyelocytic Leukemia (APL) has become the most curable subtype of acute myeloid leukemia in adults with the advent of the differentiating agents, all-trans retinoic acid and arsenic trioxide. However, Early Death (ED) remains a challenge and represents the last major obstacle to the cure of almost every patient. An overwhelming majority of ED in APL is attributable to life-threatening bleeding, a phenomenon driven by complex alterations in the coagulation system. Therefore, prompt recognition of APL and immediate initiation of All-Trans Retinoic Acid (ATRA) in emergency departments–prior to confirmation of the diagnosis–is essential. All-trans retinoic acid must be immediately available, which is often problematic since most institutions will see very few APL patients in a year and will not find it cost-effective to maintain a supply. Aggressive management of the coagulopathy and less common causes of ED, such as the differentiation syndrome and infections, is required. Since emergency departments and immediate care healthcare professionals are often the first to encounter a patient with APL, their training should include comprehensive diagnostic and initial management details. Recent data suggest that early consultation with a colleague with expertise in APL for initial hour-by-hour management is very helpful in reducing the ED rate. Tackling this last major obstacle in the cure of all patients requires providers to astutely recognize the disease and manage patients according to recommended guidelines.
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Journal of Blood & Lymph received 443 citations as per Google Scholar report
