Paediatrics & Child Health: Haematology and Oncology

Presentations

• Fever with neutropenia
• Lymphadenopathy

Conditions

• Anaemias:
  
  • haemoglobinopathy (sickle cell disease)
  • haemolytic (autoimmune)
  • nutritional (iron, folate, and vitamin B12)
  • thalassaemia (alpha and beta)
• Bleeding disorders:
  
  • disseminated intravascular coagulation haemophilia (factor VIII and IX deficiency)
• Bone marrow failure:
  
  • pancytopenia as a presenting feature of:
    
    • aplastic anaemia
    • leukaemias
    • syndromes
  • treatment-induced bone marrow suppression
• Brain tumour:
  
  • low grade glioma
  • medulloblastoma
• Disseminated intravascular coagulopathy
• Haemolytic disease of the neonate
• Late effects of cancer treatment:
  
  • cardiomyopathy
  • hearing impairment
  • hormone deficiencies
  • infertility
  • neurocognitive impairment
  • pulmonary fibrosis
  • renal impairment
• Leukaemias:
  
  • acute lymphoblastic leukaemia, precursor B- and T-cell
  • acute myeloid leukaemia
• Lymphomas:
  
  • B-cell non-Hodgkin lymphoma
  • Hodgkin lymphoma
• Mucositis secondary to cancer treatment
• Oncological emergencies:
  
  • spinal cord compression
  • mediastinal mass:
    
    • airway obstruction
    • pericardial effusion and tamponade
    • superior vena cava obstruction
  • tumour lysis syndrome (TLS)
• Solid tumours:
  
  • neuroblastoma (NB)
  • osteosarcoma
  • rhabdomyosarcoma
  • Wilms tumour
• Thrombocytopenia:
  
  • immune thrombocytopenia (ITP)

For each presentation and condition, Basic Trainees will know how to:

Synthesise

• recognise the clinical presentation
• identify relevant epidemiology, pathophysiology, and clinical science
• take a relevant clinical history
• conduct an appropriate examination
• establish a differential diagnosis
• plan and arrange appropriate investigations
• consider the impact of illness and disease on patients¹ and their quality of life

Manage

• provide evidence-based management
  For less common or more complex presentations and conditions the trainee must also seek expert opinions
• prescribe therapies tailored to patients’ needs and conditions
• recognise potential complications of disease and its management, and initiate preventative strategies
• involve multidisciplinary teams

Consider other factors

• identify individual and social factors and the impact of these on diagnosis and management

Presentations

• Cancer related pain
• Common cancer presentations

Conditions

• Anaemias:
  
  • aplastic
  • chronic disease
  • G6PD deficiency
  • lead toxicity
  • transient erythroblastopenia of childhood
• Bleeding disorders:
  
  • platelet function disorders
  • von Willebrand disease (vWD)
• Bone marrow failure syndromes:
  
  • Fanconi anaemia
• Brain tumours:
  
  • brainstem glioma
  • craniopharyngioma
  • ependymoma
  • high grade glioma
• Cancer predisposition syndromes:
  
  • Beckwith–Wiedemann syndrome (BWS)
  • hereditary retinoblastoma
  • Li–Fraumeni syndrome (LFS)
  • neurofibromatosis
  • trisomy 21
• Haematological manifestations of systemic and chronic disease:
  
  • genetic disorders presenting with haematologic or cancer manifestations
  • haemolytic anaemia of the neonate
• Iron overload:
  
  • haemochromatosis
  • transfusion induced
• Leucocyte disorders
• Leukaemias:
  
  • mature B-cell, acute
  • Philadelphia positive acute lymphoblastic leukaemia (Ph+ALL)
• Lymphomas:
  
  •  T-cell lymphoblastic lymphoma
• Neutropenia:
  
  • congenital
  • cyclic
  • transient
• Red cell structural disorders, such as hereditary spherocytosis
• Solid tumours:
  
  • Ewing sarcoma (ES)
  • germ cell tumour (non-central nervous system)
  • hepatoblastoma
  • retinoblastoma
• Splenic disorders:
  
  • asplenia
  • hypo- and hypersplenism
• Thrombosis:
  
  • cerebral venous sinus
  • deep venous
  • inherited clotting predisposition
  • intravascular device related

For each presentation and condition, Basic Trainees will know how to:

Synthesise

• recognise the clinical presentation
• identify relevant epidemiology, pathophysiology, and clinical science
• take a relevant clinical history
• conduct an appropriate examination
• establish a differential diagnosis
• plan and arrange appropriate investigations
• consider the impact of illness and disease on patients¹ and their quality of life

Manage

• provide evidence-based management
  For less common or more complex presentations and conditions the trainee must also seek expert opinions
• prescribe therapies tailored to patients’ needs and conditions
• recognise potential complications of disease and its management, and initiate preventative strategies
• involve multidisciplinary teams

Consider other factors

• identify individual and social factors and the impact of these on diagnosis and management

• Anticoagulant therapy:
  
  • actions and indications of anticoagulants, both prophylactic and therapeutic
  • adjusting therapy to achieve target ranges and monitoring therapy appropriately
  • drug interactions, adverse effects, pharmacokinetics, and monitoring of anticoagulation
  • initiation of anticoagulation with appropriate agent at appropriate dose taking patient factors into consideration, such as age and comorbid conditions
  • managing over-anticoagulation
• Coagulation physiology
• Cytotoxic drug mechanism of action and toxicity
• Genetic diseases of abnormal haemoglobin:
  
  • sickle cell disease
  • thalassaemia
• Growth factors:
  
  • erythropoietin
  • filgrastim
  • thrombopoietin receptor antagonists
• Haematopoiesis physiology
• Haemoglobin physiology, including antenatal
• Indications and complications of bone marrow transplant
• Iron, folate, and vitamin B12 intake and metabolism
• Processes of:
  
  • cell growth and ageing, cell injury, and apoptosis
  • immune evasion and immune surveillance
  • metastatic spread
  • molecular and cellular oncogenesis
• Purpose and principles of cancer staging
• Radiation therapy mechanism of action and toxicity
• Targeted anticancer therapy mechanism of action

Investigations

• Blood count
• Coagulation profile
• Haemoglobin electrophoresis
• Imaging for cancer diagnosis and staging:
  
  • chest X-ray (recognise mediastinal mass and airway compromise)
  • CT and MRI scans (recognise significant abnormalities such as
    large tumours on images)
  • PET, MIBG scintigraphy, and bone scan
• Iron studies
• Minimal residual disease (MRD) measurement for acute lymphoblastic
  leukaemia risk stratification (positive or negative reports only)
• Tumour markers:
  
  • alpha-fetoprotein (AFP)
  • catecholamine
  • human chorionic gonadotrophin (hCG)

Procedures

• Blood product transfusion:
  
  • immunoglobulin
  • platelets
  • red blood cells
• Bone marrow aspirate and trephine

• Educational and social impacts of cancer diagnosis and therapy
• Immunosuppression
• Late effects of cancer therapy and monitoring
• Mental health aspects of congenital bleeding disorders and chronic transfusion dependence
• Palliative care and end-of-life decision making
• Principles and role of transplantation
• Role of MIBG scintigraphy in the staging and response assessment of paediatric neuroblastoma