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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. ALL AML 80% 15% 2 - 6 yo 0 - 15 yo 2 - 3 yr, mild Rx 6mo, aggressive Rx 80% cure 65% cure .
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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% 2 - 6 yo 0 - 15 yo 2 - 3 yr, mild Rx 6mo, aggressive Rx 80% cure 65% cure
Acute leukaemia - diagnosis • 1. Clinical • 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 1.0 - 1000 x 109/L Neutropaenia ANC 0.5 x 109/L Anaemia Hb 7.8 g/dL Thrombocytopaenia Plt 40 x 109/L Chemistry Incerased Uric acid Increased LDH B Marrow Morphology 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 • 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 risk 60% Regimen B - Intermediate risk 30% Regimen C - High risk 10% Risk assignment 1. Age 2. WBC 3. Cytogenetics 4. Response to treatment
Treatment - MRC ALL 2003 1. Age (years) < 1 to Interfant protocol 1 - 10 to Regimen A > 10 to Regimen B
Treatment - MRC ALL 2003 2. WBC (x 109/L) < 50 to Regimen A >50 to Regimen B
Treatment - MRC ALL 2003 3. Cytogenetics Hypodiploidy to Regimen C t(9:22) to Regimen C iAMP 21 to Regimen C
Treatment - MRC ALL 2003 4. 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)