Childhood Leukaemia Aengus S. O’Marcaigh, M.D., F.R.C.P.I., F.A.A.P. Department of Paediatric Haematology &Oncology Our Lady’s Hospital for Sick Children Crumlin, Dublin 12.

Acute Leukaemia ALLAML 80%15% yo yo yr, mild Rx6mo, aggressive Rx 80% cure65% cure

Acute leukaemia - diagnosis 1. Clinical 2. Blood tests 3. Bone marrow tests

Diagnosis - Clinical History-Fatigue, bruising, bleeding (symptoms) -Bone pain, limping Examination- Pallor (signs)-Bruising -Fever / infection -Organomegaly -Lymphadenopathy -Mediastinal mass

Diagnosis - Clinical Examination - Pallor -Bruising -Fever -Organomegaly -Lymphadenopathy -Mediastinal mass

Diagnosis - Clinical Examination - Pallor -Bruising -Fever -Organomegaly -Lymphadenopathy -Mediastinal mass

Diagnosis - Clinical Examination - Pallor -Bruising -Fever -Organomegaly -Lymphadenopathy -Mediastinal mass

Diagnosis - Clinical Examination - Pallor -Bruising -Fever -Organomegaly -Lymphadenopathy -Mediastinal mass

Diagnosis - Clinical Examination - Pallor -Bruising -Fever -Organomegaly -Lymphadenopathy -Mediastinal mass

Acute leukaemia - diagnosis FBC-Leucocytosis ? WBC x 10 9 /L NeutropaeniaANC 0.5 x 10 9 /L AnaemiaHb 7.8 g/dL ThrombocytopaeniaPlt 40 x 10 9 /L ChemistryIncerased Uric acid Increased LDH B MarrowMorphology Cytochemistry Immunophenotyping Genetics

Diagnosis - Bone marrow Bone marrow diagnosis -Morphology -Cytochemistry -Immunophenotyping -Cytogenetics

Diagnosis - Bone marrow Bone marrow diagnosis -Morphology -Cytochemistry -Immunophenotyping -Cytogenetics Normal bone marrow

Diagnosis - Bone marrow Bone marrow diagnosis -Morphology -Cytochemistry -Immunophenotyping -Cytogenetics Acute Leukaemia

Diagnosis - Cytogenetics Translocation t (1;22) Infant M7 AML Very poor prognosis

Initial management 1.Pancytopaenia ? 2. Sepsis / infection 3. Mediastinal mass 4. Tumour lysis syndrome Transfusion CMV-neg, irradiated

Initial management 1. Pancytopaenia 2. Sepsis / infection 3. Mediastinal mass 4. Tumour lysis syndrome ? Uric acid / LDH Renal function Hydration Urate oxidase

Treatment ALL 2003 Regimen A-Standard risk60% Regimen B-Intermediate risk30% Regimen C-High risk10% Risk assignment1. Age 2. WBC 3. Cytogenetics 4. Response to treatment

Treatment - MRC ALL Age (years) < 1to Interfant protocol to Regimen A > 10to Regimen B

Treatment - MRC ALL WBC (x 10 9 /L) < 50to Regimen A >50to Regimen B

Treatment - MRC ALL Cytogenetics Hypodiploidyto Regimen C t(9:22)to Regimen C iAMP 21 to Regimen C

Treatment - MRC ALL Response to therapy - Day 8 bone marrow>25% blasts- B to C Day 15 bone marrow >25% blasts- A to C - Day 28 MRD (minimal residual disease)