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

Transcription

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

2 Anaemia Normal Ranges

3 Physiological Anaemia Causes Normal babies, more marked in Prematures Impaired Production Reduced Haematinics (IRON( IRON,, B12, Folate) Bone marrow failure Dyserythropoesis ie Thalassaemia Aplasia,, Leukaemia, Anaemia of chronic disease Increased Breakdown ie Haemolysis Increased Loss-unusual

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5 Iron deficiency Features Presents in Infancy and Toddlers Asians Pallor, Lethargy, Pica Infections Poor school performance Investigation Low Hb,, MCV, film,ferritin Exclude other causes of microcytosis

6 DIETRY Iron deficiency Infants and Toddlers, esp Asians fussy eaters high milk intake high requirements prematurity Malabsorption Increased loss GIT bleeding, menstruation

7 Management 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

8 Haemolysis Congenital or Aquired Intrinsic or Extrinsic Features Anaemia, Jaundice, Splenomegaly, Increased reticulocyte count Level of Hb depends on balance between Production (marrow activity and haematinic supply) and breakdown

9 Causes of Haemolysis 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

10 Hereditary Spherocytosis Autosomal dominant Chronic Anaemia, Jaundice and Splenomegaly Crises precipitated by infection Management: Folate supplements Splenectomy benefits and hazards

11 Sickle cell Anaemia Mainly African, Middle East, Asia Sickle Haemoglobins S, C, D, E Substitutions in ß globin chain Autosomal Recessive Normal Carrier/Trait AA AS, AC Homozygous Sickle Anaemia SS, SC, SD Sickle/thal Minor variants CC, DD, EE

12 Sickle cell Anaemia Heterozygotes Asymptomatic Sickle Presentation Antenatal/Newborn screening Chronic anaemia 6-8g/dl6 Crises PAIN limbs, chest or abdomen precipitated by Cold, Hypoxia, Infection, Dehydration, Acidosis Falling Hb Other types of crisis Aplastic, Splenic sequestration

13 Sickle cell Anaemia Complications Anaemia usually 6-8g/dL6 Poor growth Delayed puberty Recurrent painful crises Splenic infarction hyposplenism Infection overwhelming sepsis 10% Stroke Aseptic necrosis of femoral head Priapism

14 Prevention Sickle cell Anaemia AN diagnosis Prophylaxis Management Folate Antibiotics, Immunization Education avoid cold, infection, dehydration Self management at home Crises IV fluids, Antibiotics, Analgesia

15 Bruising and Bleeding Trauma- Accidental and Non-Accidental Vascular Platelets Coagulation system

16 Bleeding Disorders Vascular Platelets HSP, Scurvy, Erlers Danlos ITP, Leukaemia Von Willibrands Aspirin Bruising, Purpura Epistaxes, GIT bleeds, stop on pressure Bruising, Purpura Epistaxes, GIT bleeds, stop on pressure Coagulation Haemorrhagic Disease of Newborn Haemophilia Delayed bleeding Deep bleeds Muscle, joints Pressure fails to stop

17 Bleeding Disorders History 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

18 Bleeding Disorders Examination Sick or well Pattern of petechiae/purpura/bleeding Congenital Malformations Skin Splenomegaly

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

20 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

21 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

22 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

23 Causes of death in UK children Cause Boys Girls Total No. % No. % No. % Cancer Accident CNS disease Malformations etc Respiratory system Infections Cardiovascular Other Total ats/childhoodcancer 23

24 Aetiology Unknown for most children The Knudson 2 hit hypothesis Genetic Familial cancer syndromes Neurofibromatosis Wilms Twins Enviromental- Radiation Infections-HTLV, EBV

25 Incidence of common tumours

26 Childhood Cancer Survival

27 Reasons for improved survival Not just about the drugs! Aim to maximise survival and minimise side effects Referral to Regional Paediatric Oncology Centres Networks with shared care centres Large multi-centre trials Better and more individualised chemotherapy, radiotherapy and surgery Improved supportive treatment Nutrition, Antibiotics, Blood products Education of medical and nursing staff

28 Acute Side Effects Vomiting Nutrition Pancytopaenia Febrile Neutropaenia Supportive Care Circulatory support IV fluids IV broad spectrum antibiotics Blood and Platelet Transfusions

29 Psychological Family and social Long Term Side Effects Radiotherapy Growth particularly CNS RT Intellectual Endocrine Puberty Fertility Surgery-CNS and bone Second malignancies 4% Chemotherapy specific Steroids Cardiac and Renal Fertility The cost of cure

30 Lymphadenopathy Thank you for seeing this 5 year old with lump on her neck left side. The lump gets bigger and smaller but doesn t t disappear

31 Lymphadenopathy Growth of lymphoid system maximum in preschool children Physiological/Reactive Variable in size, bigger with infections May be local infection Painful, tender Mobile Pathological Hard, fixed, non tender, progressive enlargement Possible ALL, Hodgkins,, and Non Hogkins Lymphoma May need FBC, Monospot,, Chest XR, Mantoux Distinguish by biopsy

32 Acute Lymphoblastic Leukaemia Most common malignancy in childhood Peak age 4-7yrs4 Prognosis 80% cure Clinical Features Anaemia Infection Bleeding Bone pain Lymphadenopathy Hepatosplenomegaly

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

34 ALL - Treatment Good Prognostic factors Age Female WCC<50 No CNS disease Classified also on cell type: Common, B and T cell Allocated into risk groups based on prognostic features Treatment escalated Poor initial response Presence of Minimal Residual Disease 4 main treatment phases: Induction Consolidation and CNS treatment Intensification Maintenance Girls 2 yrs Boys 3 yrs

35 Brain Tumours Most common solid tumour Peak age yrs Usually primary and infratentorial Prognosis variable usually about 70% High residual morbidity

36 Brain Tumour Symptoms and Signs Raised Intracranial Pressure Early morning/night Headache Vomiting Papillodema Drowsiness- late School Failure- subtle Focal signs/symptoms Ataxia, slurred speech Hemiplegia Diplopia Convulsions Precocious Puberty

37 Brain Tumour- Sites Juvenile Astrocytoma Craniopharygioma Primitive Neuroectodermal Tumours: (Ependymoma) (Medulloblastoma) Brainstem Glioma Cervical Glioma

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39 Brain Tumour Treatment Early diagnosis and urgent referral Emergency treatment Dexamethasone Surgery - can be difficult Radiotherapy- side effects Chemotherapy-limited value

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41 Thalassaemia Reduced globin chain synthesis Normal α2 β2 β Thal Hb F (α( and γ) ) and Hb A2 (α( and δ) α Thal 4 alles loss 1 or 2 asymtomatic loss 3or 4 Hb H β4

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

43 β Thalassaemia Management Genetic Counselling, AN diagnosis Regular blood transfusion Complications of Iron overload Liver, Heart, Pancreas, Endocrinopathy Iron chelation Bone Marrow Transplantation

44 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

45 Non Hodgkin s 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

46 Hodgkin s 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

47 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

48 Wilm s Tumour Renal tumour- Nephroblastoma Toddlers 2-5yr 2 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