Day 2: Acute lymphoblastic leukaemia – risk factors and prognosis

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 We suspect that Hamish has ALL – how do we confirm this, and more importantly, what is his prognosis?

View all our Acute Lymphoblastic Leukaemia Week posts

What are the initial investigations?

  • 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.

What are the risk factors for ALL?

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

What are good prognostic factors for ALL?

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.

What do remission, relapse, and cure mean?

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.

Using the most up to date study outcomes, the 5 year survival is 85%

Once the child gets further through the initial treatment and is given a standard risk, then the 5 year survival is 97%

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About 

A Paediatric Trainee based in Queensland, Australia, Henry is passionate about Adolescent Medicine & General Paediatrics, with a strong interest in Medical Education & Clinical Teaching. An admitted nerd & ironman with a penchant for Rubik's Cubes & 'Dad jokes'.

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