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Paediatric Haematology and Oncology

Paediatric Haematology and Oncology. All in One hour! Dr Nigel Coad. Anaemia Normal Ranges. Anaemia Causes. Physiological Impaired Production Bone marrow failure Dyserythropoesis ie Thalassaemia Aplasia, Leukaemia, Anaemia of chronic disease Reduced Haematinics (Iron, B12, Folate)

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Paediatric Haematology and Oncology

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  1. Paediatric Haematology and Oncology All in One hour! Dr Nigel Coad

  2. Anaemia Normal Ranges

  3. AnaemiaCauses • Physiological • Impaired Production • Bone marrow failure • Dyserythropoesis ie Thalassaemia • Aplasia, Leukaemia, • Anaemia of chronic disease • Reduced Haematinics (Iron, B12, Folate) • Increased Breakdown ie Haemolysis • Increased Loss-unusual

  4. Iron deficiency Features Presents in Infancy and Toddlers Asians Pallor, Lethargy, Pica Risk of Infection Poor school performance Functional murmer Investigation Low Hb, MCV, Ferritin Exclude other causes of microcytosis

  5. Iron deficiency • Dietary • Infants and Toddlers, esp Asians • fussy eaters • high milk intake • high requirements • prematurity • Malabsorption • Increased loss • GIT bleeding, menstruation

  6. Treatment of Iron deficiency • Advice re Diet • Give Iron supplements and continue • Follow up to check response • Investigate underlying pathology • malabsorption • blood loss • Rarely need transfusion or parenteral Iron

  7. Haemolysis • Congenital or Aquired • Intrinsic or Extrinsic Features Anaemia, Jaundice, Splenomegaly, Bilirubinuria Increased Retics Level of Hb depends on balance between Production (marrow activity and haematinic supply) and breakdown

  8. Intrinsic Membrane defects Hereditary Spherocytosis, Elliptocytosis Abnormal Haemoglobin Sickle cell Thalassaemia Enzyme defect G6PD Pyruvate Kinase deficiency Extrinsic Haemolytic Disease of Newborn Autoimmune Drug induced Haemolytic Uraemic syndrome Causes of Haemolysis

  9. Hereditary Spherocytosis • Anaemia, Jaundice and Splenomegaly • Autosomal dominant • Crises precipitated by infection • Folate supplements • Splenectomy • benefits and hazards

  10. Sickle cell Anaemia • Mainly African, Middle East, Asia • substitutions in ß globin chain • Autosomal Recessive • Normal AA • Carrier/Trait AS, AC • Sickle Anaemia • SS, SC, SD • Sickle Thal • Variants CC, DD, EE

  11. Sickle cell Anaemia • Heterozygotes Asymptomatic • Sickle Presentation • screening • anaemia 6-8g/dl • Crises PAIN limbs, chest or abdomen precipitated by Hypoxia, Infection, Dehydration, Acidosis • Other types of crisis • Aplastic, Splenic sequestration

  12. Sickle cell Anaemia Complications • Anaemia • Splenic infarction hyposplenism • Infection overwhelming sepsis • 10% Stroke • Aseptic Necrosis of Femoral Head • Poor Growth, delayed puberty • Priapism • Leg Ulcers

  13. Sickle cell AnaemiaManagement • Prevention • AN diagnosis • Prophylaxis • Folate • Antibiotics, Immunization • Avoid Cold infection dehydration • Crises • IV fluids, Antibiotics, Analgesia

  14. Bruising and Bleeding • Trauma- Accidental and Non-Accidental • Vascular • Platelets • Coagulation system

  15. Bleeding Disorders

  16. Bleeding DisordersHistory • Pattern of Bruising/bleeding • Recurrent epistaxes common • Easy bruising non specific • Most children have limb bruises • Incidents recalled • cicumcision, tonsillectomy, dental extraction • Family history • Drugs • Recent Infection

  17. Bleeding DisordersExamination • Sick or well • Pattern of petechiae/purpura/bleeding • Congenital Malformations • Skin • Splenomegaly

  18. Investigations • Platelet Count+ FBC • PTT • Factors XII, XI, X, IX, VIII, V, II • PT • Factors X, VII, V, II • Bleeding Time

  19. Immune Thrombocytopaenic Purpura (ITP) • Usually young children • Post viral • Recover spontaneously- weeks to months • Rarely dangerous, but looks dramatic • Nothing else abnormal • No spleen, anaemia or neutropaenia • No treatment • Rarely need steroids, Immunoglobulin or Splenectomy

  20. Haemophilia • Deficiency of Factor VIII, Abnormal PTT • X linked recessive- boys • Prolonged bleeding • Muscle bleeds • Joint bleeds > Arthritis and deformity • Treatment Factor VIII • Complications of treatment

  21. Cancer in Children Incidence 1500 children yearly in UK 150 yearly in W Midlands Prevalence 1 in 600 children ie approximately 100 children in Coventry

  22. Aetiology Unknown for most children The Knudson 2 hit hypothesis Genetic Familial cancer syndromes Neurofibromatosis Wilms Twins Enviromental Radiation Infections

  23. Incidence of common tumours

  24. Improved Survival

  25. Reasons for improved survival Referral to Regional Paediatric Oncology Centres What are the benefits? Improved understanding of disease and complications Better and more individualised chemotherapy, radiotherapy and surgery Large multi-centre trials Improved supportive treatment Education of medical and nursing staff Improved shared care with joint care standards

  26. Long Term Side Effects Psychological Family and social Growth particularly CNS RT Endocrine Puberty Fertility Intellectual Second malignancies Chemotherapy specific

  27. Acute Lymphoblastic Leukaemia Most common malignancy Peak age 4-7yrs Prognosis 80% cure Good Prognostic factors Age 2-10 Female WCC<50 No CNS disease Classified also on cell type Common, B and T cell

  28. ALL- Clinical Features Anaemia Infection Bleeding Bone pain Lyphadenopathy Hepatosplenomegaly

  29. ALL- Making the diagnosis Blood count Anaemia WCC up or down Neutropaenia Thrombocytopaenia Blast cells Marrow LP

  30. ALL - Treatment Stratified into risk groups A B C Induction Consolidation and CNS treatment Intensification Maintenance Girls 2 yrs Boys 3 yrs

  31. Brain Tumours Most common solid tumour Peak age 5-9 yrs Usually primary and infratentorial Prognosis variable usually about 50%

  32. Brain Tumour Symptoms • Raised Intracranial Pressure Early morning/night Headache Vomiting Drowsiness School Failure • Focal signs/symptoms Ataxia, slurred speech Hemiplegia Diplopia Convulsions Precocious Puberty

  33. Brain Tumour- Sites Juvenile Astrocytoma Craniopharygioma Primitive Neuroectodermal Tumours: (Ependymoma) (Medullobastoma) Brainstem Glioma Cervical Glioma

  34. Brain Tumour Treatment Surgery - can be difficult Radiotherapy- side effects Chemotherapy-limited value

  35. Thalassaemia • Reduced globin chain synthesis • Normal 2 2 •  Thal Hb F ( and ) and Hb A2 ( and ) •  Thal 4 alles • loss 1 or 2 asymtomatic • loss 3or 4 Hb H 4

  36.  Thalassaemia •  Thalassaemia Minor • asyptomatic • Mild anaemia, low MCV, Raised Hb A2 •  Thalassaemia Major • Progressive Severe Anaemia, low MCV, Hb F and A2 • Jaundice (splenomegaly) • Failure to thrive • Skeletal Deformity • Delayed puberty • Death early teens/adulthood

  37.  ThalassaemiaManagement • Genetic Counselling, AN diagnosis • Regular blood transfusion • Complications of Iron overload • Liver, Heart, Pancreas, Endocrinopathy • Iron chelation • Bone Marrow Transplantation

  38. Von Willibrand’s Disease • Autosomal dominant • Less severe than haemophilia, possibly 1% of population • VW Factor • secreted by endothelium /platelets • Carrier for FVIII:C • Facilitates platelet adhesion • Tests • Prolonged Bleeding time, PTT, • Low FVIII:C, vWF, Ristocetin Cofactor Assay • Treatment • DDAVP, Factor VIII

  39. Non Hodgkin’s Lymphoma Tumour of lymphoid tissue 70% cure Prognosis depends on stage T and B cell Types Lymphadenopathy Chest mediastinal nodes, pleural effusions Hepatosplenomegaly Ascites Treatment Chemotherapy

  40. Hodgkin’s Disease Tumour of Lymphoid tissue Prognosis 50-95% depending in stage Most children present early and have excellent prognosis Cervical lymphadenopathy Night sweats, fever, weight loss Biopsy Staging important- CT Chest and Abdomen Combination chemotherapy- most children Radiotherapy- for localised or advanced disease

  41. Neuroblastoma Tumour of sympathetic chain Arises in chest or abdomen,adrenals rarely in neck or eye Highly malignant spread to bone, liver and locally Prognosis depends on stage,most present late Typically about 25% survival Unwell toddler bone pain, abdominal swelling Massive abdominal mass Diagnosis Abdominal ultrasound Urine Catecholamines Combination chemotherapy, surgery and radiotherapy

  42. Wilm’s Tumour Renal tumour- Nephroblastoma Toddlers 2-5yr old Excellent prognosis >80% cure Most localised to kidney Spread locally,invades veins, rarely to lungs Presents as a well child with abdominal mass, pain or haematuria Treatment depends on stage Surgery or chemotherapy occasionally radiotherapy

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