Clinical characteristics of acute promyelocytic leukemia manifesting as early death

Acute promyelocytic leukemia (APL) is currently considered to be a highly curable disease. However, early death (ED) remains a major cause of treatment failure in APL. The purpose of this study was to retrospectively review the morphological, immunophenotypic and molecular characteristics of 26 patients with APL resulting in ED. It was observed that elevated white blood cell (WBC) counts, lower fibrinogen concentrations, morphological variant M3v, CD34(+) and the short form (S- or bcr3 form) of the PML-RARα transcript were significantly associated with ED, mainly due to cerebral hemorrhage. Admission on weekends or holidays without immediate diagnosis or prompt administration of treatment for APL resulted in intracranial bleeding and was the major cause of ED. Therefore, it is recommended that APL and coagulopathy management treatments are promptly initiated only upon morphological and clinical suspicion of APL on admission, in order to reduce the risk of severe bleeding and lower the rate of ED.