Acute promyelocytic leukaemia in children

Abstract

Acute promyelocytic leukaemia is characterized by coagulopathy, distinctive haematological findings and diagnostic (15;17) translocation. We reviewed all our patients with acute promyelocytic leukaemia managed between 1983-94. Diagnosis was based on blood and marrow findings according to French-American-British classification. There were 8 patients (5 males, 3 females), accounting for 44.4% of children with acute myeloid leukaemia seen in the same period. Bleeding diathesis was common on presentation. Coagulopathy was evident in four patients. Three patients failed to achieve remission after induction. Two of the three died of disease progression while one went into complete remission after allogeneic marrow transplant but died of bronchiolitis obliterans 1 year later. Five patients achieved complete remission. Four of the five (80%) relapsed within the first year (one died, one defaulted, one currently being reinduced, one survived for more than 2 years after marrow transplant from a matched unrelated donor). The overall survival was 37.5%. All-trans retinoic acid was given to three patients, all resulted in remissions without adverse reactions.