Canterbury DHB

Context

Acute Promyelocytic Leukaemia

This section was reviewed by Dr Ruth Spearing in October 2018. The next review date is October 2020.

In This Section

Overview

Clinical Features

Laboratory Diagnosis

Prognosis and the Decision to Treat

Pre-Treatment Investigations

Management

AML19 APML

Treatment of non-trial patients

Overview

Clinical Features

These are essentially the same as for other forms of AML except that the haemorrhagic tendency may be very severe and can be made temporarily worse by initial therapy. This is due to a combination of thrombocytopenia and DIC/increased fibrinolysis.

Laboratory Diagnosis

The diagnosis may be suggested by immunophenotyping (often CD34 and HLADR negative and CD9, 13, 33 positive). The diagnosis however requires the demonstration of the t(15;17) translocation by FISH or cytogenetics, and / or the demonstration of PML-RARα by DNA analysis.

AML with cup-like blasts is classically also CD34 and HLA DR neg and may cause confusion on morphology. It may also be associated with DIC and is often associated with a high white count. It is important to distinguish this form of AML from APML. See Jalal S. et al, BJH 2010 148: 182.

For a full account of the laboratory findings of APML, refer to the WHO Classification of Tumours – Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues, 2008, pp 112 - 114.

Prognosis and the Decision to Treat

Prognosis

APML is included in the WHO 2016 classification of “AML with recurrent cytogenetic changes”. The prognosis for APML is better than other forms of AML with a CR rate of more than 90% and overall survival of around 80% at 5 years. The main risk of serious complications is due to bleeding, which arises at presentation and during the initial 5-7 days of treatment.

The Decision to Treat

In general, treatment should be offered to all patients with APML as soon as the t(15;17) and/or the PML-RARα defect has been demonstrated or the morphology indicates these changes are almost certain to be present.

Intensive supportive treatment to prevent bleeding plus tretinoin (ATRA)—with or without chemotherapy—is indicated in virtually all patients, regardless of age. This is because of the excellent results that are usually obtained.

In Christchurch, patients with APML will be offered treatment within the AML19 trial. See section on AML19 APML and Clinical Trials page on the Intranet. Alternatively you can phone the Clinical Trials Co-ordinator (ext: 80377).

Pre-Treatment Investigations

See the Investigations section under AML.

Management

About this Canterbury DHB document (4299):

Document Owner:

Ruth Spearing (see Who's Who)

Issue Date:

October 2018

Next Review:

October 2020

Keywords:

Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer

Topic Code: 4299