Acute lymphoblastic leukaemia – risk factors and prognosis

• Repeat FBC & send group & hold; coagulation profile; blood cultures if febrile; electrolytes, including PO4-, Mg+, Ca++ as high WCC at risk of tumor lysis syndrome; liver function tests; Hep B, C, HIV, EBV, CMV, herpes simplex, HHV6, syphilis & toxoplasma serology
• Blood film must be reviewed & reported by a consultant haematologist
• ECG – sinus tachycardia, normal axis
• Chest radiograph – to check for mediastinal mass
• Urinalysis
• Official height & weight – for chemotherapy & body surface area calculations (standard scales/measure, sighted by two staff)
• Pregnancy test in females of childbearing age

Once clinically stabilised (including meeting minimum platelet counts), they will have a GA lumbar puncture (usually with intrathecal chemotherapy) a bone marrow aspirate, and if there is no contraindications, insertion of a tunnelled central line.

• Family history
• Immunosuppression
• Alkylating agents (more commonly linked to AML rather than ALL)
• Trisomy 21
• Neurofibromatosis
• Ataxia telangiectasia
• Bloom syndrome

1. Age  >1yo and <10yo at diagnosis

2. White cell count <50×10⁹/L at presentation

3. No testicular involvement at presentation

4. Not a child with Down Syndrome

5. No prior steroid exposure – this is important, as steroids are themselves chemotherapeutic and can put a child into remission as a single agent. If there has been a history of URTI or wheeze, they may have been prescribed (or been given a sibling’s) steroids. There are reports of spontaneous tumour lysis syndrome in undiagnosed patients as a result of steroids. Steroid exposure will move the child to a high-risk protocol.

6. No CNS disease – established with first CSF examination

In recent years, cytogenetics & minimal residual disease (MRD) has added a further layer to prognosis and treatment. This analysis requires CSF & bone marrow samples.

For diagnosis – a patient has to have over 25% blasts in the peripheral blood film

For remission – a patient has to have <5% blasts in the peripheral blood film

For cure – a patient has to have no evidence of leukaemia over 5 years from diagnosis

Bone marrow is the most common site for relapses and 10% of relapses are central nervous system only. In boys, testes are a known site of relapse and present as a hard testicular lump – so make sure you examine the tests during follow-up appointments.