Acute promyelocytic leukaemia:a review

Abstract

Acute promyelocytic leukaemia (APL) is characterised by the fusion gene transcript PML-RAR-alpha and is now the most frequently curable acute leukaemia in adults if promptly diagnosed and adequately treated. The clinical presentation is associated with a haemorrhagic diathesis and the blasts almost always have Auer rods. Poor prognostic factors include older age, elevated white blood cell count, low platelet count, and CD56 expression. The introduction of all-trans retinoic acid (ATRA), which leads to the differentiation of leukaemic blasts into mature granulocytes has been the major breakthrough in the treatment of APL. Induction treatment with concurrent ATRA and chemotherapy leads to a rapid resolution of the characteristic life-threatening coagulopathy, high complete remission rates and excellent survival rates, compared to chemotherapy alone. However, treatment with ATRA is associated with the retinoic acid syndrome (RAS), which is a major toxicity and may lead to mortality. The role of cytarabine as a part of initial induction regimen remains unclear. After achievement of complete remission (CR), there is a definitive role of maintenance therapy with ATRA with or without low-dose chemotherapy. In relapsed patients, arsenic trioxide is considered the treatment of choice. However, the best postremission treatment for patients with second CR remains unknown. With the continued improvement in the field of stem cell transplantation, it may play an important role in the few patients with relapsed/refractory disease or those in second CR.