Acute Promyelocytic Leukaemia in Adults at Chris Hani Baragwanath Academic Hospital

Background: Acute Promyelocytic Leukaemia (APL) is a subtype of Acute Myeloid Leukaemia (AML). APL is a medical emergency due to its high early mortality rate, most commonly due to bleeding complications resulting from an associated coagulopathy. However, APL is eminently treatable. Aim: The aim of the study was to review the clinical profile as well as the outcome of adult patients presenting with APL, to a tertiary, public sector hospital (Chris Hani Baragwanath Academic Hospital -CHBAH) in Soweto, Johannesburg, South Africa. Patients and Methods: This study entailed a retrospective review of 79 evaluable patients with APL, seen over a 25 year period. Results: Of the 79 evaluable patients, there were 39 males (49.37%) and 40 females (50.63%), with a female to male ratio of 1.03:1. The median age at presentation was 32 years. APL coagulopathy was evident at presentation, with 82.05% of the patients presenting with bleeding and 4.29% presenting with thrombosis. Laboratory evaluation revealed that 93.42% of the patients had anaemia and 97.37% of the patients had thrombocytopenia, while 63.93% of the patients had evidence of a DIC (disseminated intravascular coagulopathy). The Sanz risk score indicated high-risk APL in 26.32% of the patients. HIV seropositivity was present in 17.72% of the patients. After excluding patients with “early mortality’, the median survival was 43.7 months. Conclusion: APL most commonly presents with bleeding manifestations, anaemia, thrombocytopenia and DIC, which contribute to a high “early mortality’ rate. APL is eminently treatable with appropriate supportive care and specific therapy (including ATRA – all trans retinoic acid, arsenic trioxide and chemotherapy). Therefore, early diagnosis and timeous referral of APL patients is of paramount importance in order to decrease the high early mortality and improve the overall outcome of patients harbouring the disease.