How I treat children and adolescents with acute promyelocytic leukaemia.

Abstract:

:Acute promyelocytic leukaemia (APL) is a rare subtype of acute myeloid leukaemia. The outcome of paediatric APL has improved substantially over the past 20 years; cure rates above 80% are expected when all-trans retinoic acid (ATRA) is given with anthracycline-based regimens. The presenting features of paediatric APL may include severe bleeding and thrombotic complications, which contribute to the high early death rate. The incidence of leucocytosis and the microgranular subtype is greater in paediatric than adult APL, and children experience greater ATRA-related toxicity. It is crucial to begin ATRA therapy and intensive platelet and fibrinogen replacement on first suspicion of APL. Recent risk-adapted therapeutic trials have shown that patients at greater risk of relapse benefit from the introduction of high-dose cytarabine during consolidation. Combination therapy with ATRA and arsenic trioxide provides very effective frontline treatment and may reduce the need for subsequent anthracycline therapy.

journal_name

Br J Haematol

authors

Abla O,Ribeiro RC

doi

10.1111/bjh.12584

subject

Has Abstract

pub_date

2014-01-01 00:00:00

pages

24-38

issue

1

eissn

0007-1048

issn

1365-2141

journal_volume

164

pub_type

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