We suspect that Hamish has ALL – how do we confirm this, and more importantly, what is his 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
- 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
- Alkylating agents (more commonly linked to AML rather than ALL)
- Trisomy 21
- 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.
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%