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Chapter: Paediatrics: Oncology

Paediatrics: Acute lymphoblastic leukaemia

This is the most common malignancy in childhood. It arises from malig-nant proliferation of ‘pre-B’ (common ALL) or T-cell lymphoid precursors.

Acute lymphoblastic leukaemia


This is the most common malignancy in childhood. It arises from malig-nant proliferation of ‘pre-B’ (common ALL) or T-cell lymphoid precursors. The cause is unknown, but in a minority it is associated with chromoso-mal aberrations. Possible links to patterns of childhood infection acting as a trigger have been hypothesized.

·  ALL accounts for 25% of all childhood malignancies.


·  Commonly presents in young children aged 2–6yrs.




Typically with a short history (days or weeks), and with symptoms and signs reflecting pancytopenia, bone marrow expansion, and lymphadenop-athy. Includes petechiae, bruising, pallor, tiredness, bone/joint pain/swell-ing, limp, lymphadenopathy, airway obstruction, and pleural effusion.


Specific diagnostic tests


·  Bone marrow: morphology; immunophenotype; cytogenetics.


·  CSF for cytospin (CNS rarely involved at first diagnosis).


·  Clinical examination of testes in boys for inappropriate swelling.


·  CXR for mediastinal mass.


Outline of ‘standard’ treatment


Induction (4wks)


·  Steroids (dexamethasone or prednisolone) throughout induction


·  Weekly IV vincristine


·  IM L-asparaginase (e.g. 9 doses in 3wks or 2 doses of pegylated asparaginase)


·  IV daunorubicin (2–4 doses, in intermediate and high risk cases)


·  Intrathecal (IT) methotrexate (day 18)


Note Tumour lysis syndrome is a significant risk Consolidation CNS-directed therapy


·  Low risk cases: 4-weekly doses of IT methotrexate and continuous oral mercaptopurine.


·  Higher risk cases: add IV cyclophosphamide, cytarabine.


·  CNS-radiotherapy only for CNS +ve cases




Continuation treatment for at least 2yrs (3yrs for boys)


·  Daily 6-mercaptopurine (6MP), weekly oral methotrexate (doses titrated according to blood count)


·  4-weekly vincristine IV bolus and 5-day pulses of oral dexamethasone


·  12-weekly IT methotrexate


Intensive blocks of chemotherapy


One or two blocks of 8wks duration, interrupting 1st year of mainte-nance. Combinations of oral steroid, vincristine, doxorubicin, cyclophos-phamide, cytarabine, and L-asparaginase



Overall survival is approximately 80% with current treatment. Adverse prognostic factors include:

·Male gender.


·Age <2yrs or >10yrs.


·High WCC at diagnosis.


·Unfavourable cytogenetics: Philadelphia chromosome—t(9;22); MLL gene rearrangements (e.g. t(4;11) in infants); AML1 amplification;


·Poor response to induction and failure to remit by day 28.


·High level of minimal residual disease (MRD) at 28 days.


Mature B-cell ALL (Burkitt’s type, L3 morphology)


Once considered a high-risk group, outlook is similar to that in stand-ard risk ALL now that patients are treated with intensive chemotherapy according to strategy for B-cell non-Hodgkins lymphoma.


Relapsed ALL


Extramedullary relapse (mainly CNS, testes) may present without bone marrow disease. Treatment is stratified according to risk factors, which include:

·Time from first diagnosis (risk reduces with time).


·Extramedullary relapse (lower risk, particularly if isolated).


·Minimal residual disease (MRD) status after re-induction (–ve status reduces risk).




·Intensive re-induction and consolidation for all risk groups.


·Low risk: 2yrs of continuing conventional chemotherapy.


·High risk: BMT allograft.


·Intermediate risk: the role of BMT in this group is unclear; it may be based on minimal residual disease and/or availability of matched donor.


·Radiotherapy for extramedullary disease: given as a boost for those receiving total body irradiation (TBI) for BMT.




Long-term survival varies: 10–90% depending on risk (e.g. 90% in those with isolated extramedullary relapse more than 2yrs off treatment).


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