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Chapter: Paediatrics: Oncology

Paediatrics: Acute myeloid leukaemia

Acute myeloid leukaemia (AML) accounts for 75% of all childhood malig-nancies and <20% of all acute leukaemias.

Acute myeloid leukaemia


Acute myeloid leukaemia (AML) accounts for 75% of all childhood malig-nancies and <20% of all acute leukaemias. It is also known as acute non-lymphoblastic leukaemia (ANLL). AML results from malignant prolifera-tion of myeloid cell precursors. AML can be subdivided morphologically using the French–American–British (FAB) classification system:

·  M1: AML without maturation.


·  M2: AML with maturation.


·  M3: acute promyelocytic leukaemia (PML).


·  M4: acute myelomonocytic leukaemia with eosinophilia (M4Eo).


·  M5: acute monocytic/monoblastic leukaemia.


·  M6: acute erythroleukaemia.


·  M7: acute megakaryocytic leukaemia.




·  Symptoms and signs of bone marrow replacement.


·  Lymphadenopathy less prominent than in ALL.


·  Intrathoracic extramedullary disease less common than in ALL.


·  M3 may present with coagulopathy from proteolytic enzyme activity.


·  Solid deposits (chloroma) occasionally seen in M2, M4, or M5.




Cytogenetic analysis shows characteristic abnormalities:

·  M1 and M2 AML: t(8;21) translocation observed in 15% of all cases.


·  M3 AML: t(15;17) translocation observed in 100% of cases;


·  M4Eo: inv(16) frequently observed.


These translocations are regarded as good prognostic indicators. Other complex karyotypes are associated with poor risk.




In AML prolonged continuation therapy is not used:

·  4 courses intensive myeloablative chemotherapy. The role of the gemtuzumab or Myelotarg (a monoclonal antibody directed against CD33) given alongside chemotherapy is being explored in the context of clinical trials.


·  PML: all-trans retinoic acid given in induction, before chemotherapy, improves survival.


·  High risk cases, including those who fail to achieve complete remission

after 2 courses, are usually offered BMT in first remission.



Overall survival is >60%.


Relapsed AML

All cases require BMT after intensive re-induction, usually in conjunction with ‘FLAG’ or ‘FLAG-Ida’ regimen (i.e. fludarabine, ara-C, and G-CSF sup-port +- idarubicin).


Leukaemia and Down syndrome


The risk of developing acute leukaemia is increased 20–30 times, com-monly either a pre-B (common) ALL or AML (especially M7). Response to chemotherapy is good and better relapse-free survival is found in those with AML. Children with Down syndrome-associated leukemia experi-ence more complications of treatment.


Other genetic conditions predisposing to AML


·Fanconi syndrome.


·Bloom syndrome.


·Ataxia telangiectasia.


·Kostmann’s syndrome.


·Diamond–Blackfan syndrome.




·Turner’s syndrome.




·Incontinentia pigmenti.


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