HaemOnc Flashcards
Define leukaemia
Malignant disorder of the bone marrow
- commonest cancer seen within paediatric population
- most common form is acute lymphoblastic leukaemia (ALL)
Epidemiology of leukaemia
Incidence of ALL peaks between 2 and 5 years of age
Males more commonly affected
Genetic diagnoses (trisomy 21) at an increased risk
Pathophysiology of ALL
Multifactorial condition - infection, genetic predisposition and environmental exposure
Disruption in the regulation and proliferation of lymphoid precursor cells in bone marrow
- excessive production of immature blast cells
- drop in numbers of functional RBC, WBC and platelets
Clinical features of ALL
Anaemia - lethargy, pale
Thrombocytopenia - early bruising/bleeding
Leukopenia - fevers, infections
Bone pain - due to increased pressure from hyperplastic marrow
Non-specific symptoms of malignancy - weight loss, malaise
Ddx of ALL
Bruising
- immune thrombocytopenia - low platelets but normal RBC/WBC
- trauma - hx of injury
- non-accidental injury - normal bloods with no hx of injury
Recurrent infections
- immune deficiency - long standing hx of unusual/severe infections, failure to thrive, chronic diarrhoea
Lymphadenopathy
- reactive lymphadenopathy - hx of recent infections
CNS symptoms
- infection
- raised intra-cranial pressure
Pancytopenia
- other malignant processes into bone marrow
- aplastic anaemia
Investigations of ALL
FBC - pancytopenia - anaemia - thrombocytopenia - significant lymphocytosis Blood film - presence of blast cells CXR - exclude mediastinal mass Bone marrow aspirate/trephine - confirms diagnosis Lumbar puncture - check for CNS involvement
Risk scoring for ALL
Age
- children between 1-10 years old have better prognosis
WCC > 50
- at presentation gives poorer prognosis
Gender
- females have better prognosis
CNS involvement
- presence of blasts within CSF a/w poorer prognosis
Leukaemia characteristics
- morphology and cytology influences prognosis
Mx of ALL
Immediate
- hyperhydration - to prevent hyperviscosity if have very high WCC
- steroids - if risk of airway compromise due to mediastinal mass
- abx - if infection
Definitive
- UKALL 2011 protocol
- chemotherapy - IV, oral and intra-thecally (into CSF)
- blood products - RBC, platelets
- prophylactic anti-fungal therapy
Prognosis of ALL
90% of children survive
Should have regular follow-up and assessment for 5 years then yearly review
Complications of ALL
Infertility
Avascular necrosis
Peripheral neuropathy
Anxiety
Define lymphoma
Malignancy of the lymphatic system
- Hodgkin’s
- non-Hodgkin’s
Epidemiology of lymphoma
Over 10% of childhood cancers
- more commonly seen in boys
- more common in older children
- just over half childhood lymphoma are non-Hodgkin
Pathophysiology of lymphoma
Multifactorial - infection, genetic and environmental exposures
Risk factors for lymphoma
Epstein-Barr virus
Immunosuppression
Other cancers
Clinical features of lymphoma
Visible or palpable mass - non-tender lymphadenopathy - mediastinal lymphadenopathy - cough, wheeze, SVCO History of B symptoms - weight loss - nights sweats - fevers
Differential diagnosis for lymphoma
Reactive lymphadenopathy - hx of recent infection Lymphadenitis - tender and fluctuant lymph nodes Leukaemia - lymphadenopathy associated with signs and symptoms of anaemia/thrombocytopenia
Ix for lymphoma
FBC - infection
U+Es - tumour lysis syndrome due to rapid cell turnover
LDH elevated
USS - identify other nodes and assists with biopsy
CXR - mediastinal involvement
Lymph node biopsy - definitive diagnosis
Risk scoring for lymphoma
Stage 1 - disease present in a single lymph node or organ
Stage 2 - disease present in 2 or more groups of lymph nodes or organs on the same side of the diaphragm
Stage 3 - disease present in lymph nodes or organs on both sides of the diaphragm
Stage 4 - diffuse involvement of lymph nodes and organs - liver and bone
Mx of lymphoma
Immediate
- mediastinal mass - high dose steroids and airway support
- SVCO - stenting of veins
- tumour lysis syndrome - hyperhydration, allopurinol or rasburicase
Definitive
- chemotherapy and possibly radiotherapy
Prognosis of lymphoma
Majority of children with recover
- Hodgkin’s lymphoma more favourable prognosis than non-Hodgkin’s
Complications of lymphoma
Tumour lysis syndrome
- rapid lysis of tumour cells releases large amounts of phosphorus, potassium and calcium
- can lead to kidney damage
- most likely to occur when chemotherapy first commenced
Neutropenia
Alopecia
Sub-fertility
Define nephroblastoma
Wilm’s tumour
Most common renal tumour affecting children
Usually occurs unilaterally
- can be bilateral
Epidemiology of nephroblastoma
80 children per year in the UK
Presents at pre-school age
Slight female predominance
Pathophysiology of nephroblastoma
Many tumours arise from nephrogenic rests
- embryonal remnant seen in around 1% of infants at birth
Genetics
- mutations of common tumour suppressor genes
Risk factors for nephroblastoma
Genetic and overgrowth syndromes
- WAGR - Wilm’s tumour, Aniridia, Genitourinary malformation and Retardation
- Denys-Drash
- Beckwith-Wiedemann
Clinical features of nephroblastoma
Abdo mass - incidental finding or by parents on otherwise well child Abdo swelling, abdo pain Fever Haematuria
Ddx for nephroblastoma
Polycystic kidney disease Hydronephrosis Neuroblastoma - abdo mass in pre-school aged child - hypertension, abdo pain and fever - periorbital ecchymosis
Ix for nephroblastoma
Urine dip - haematuria
Uss
CT/MRI - more detailed and for staging
Biopsy for definitive diagnosis
Staging of nephroblastoma
1 - tumour confined to kidney and can be completely removed with surgery
2 - tumour has spread beyond kidney but can be completely removed with surgery
3 - tumour cannot be completely removed with surgery as has spread to neighbouring lymph nodes or ruptured before/during surgery
4 - distant metastases - most commonly lungs
5 - bilateral tumours
Mx of nephroblastoma
Supportive care - ensure nutrition and hydration optimised
Stage 1 and 2 = soley surgery
- preserve renal function whilst removing malignant lesion
- commonly nephrectomy
Chemo - reduced volume of malignant tissue before surgery or treat any areas of malignant disease not removed by surgery
Prognosis of nephroblastoma
Good even for metastatic disease
- 85% cured
Complications of nephroblastoma
Single functioning kidney
Cardiotoxicity due to chemotherapy
Risk factors for iron deficiency anaemia
Poor dietary intake
Chronic inflammatory conditions
Menorrhagia
Lower socio-economic background
Clinical features for iron deficiency anaemia
Often incidental finding - asymptomatic Fatigue Malaise Poor exercise tolerance Irritability Headache Syncope Pallor Angular stomatitis Glossitis Nail changes Poor weight gain Tachycardia Should be no hepato/splenomegally
Ix for iron deficiency anaemia
Reduced Hb Reduced MCV Reduced ferritin Increased total iron-binding capacity Reduced transferrin
Mx of iron deficiency anaemia
Oral iron replacement - until Hb normal then 3 more months Dietary advice - green veg and red meat - better absorbed with vit C Blood transfusion rarely indicated Failure to respond to oral iron - further investigation - malabsorption - blood loss
Causes of haemolytic anaemia
G6PD deficiency
- cells susceptible to damage from oxidation
- Heinz bodies seen on blood film
Hereditary spherocytosis
- membrane defects make RBCs spherical
- spherocytes seen on blood film
Autoimmune haemolytic anaemia
- antibodies to RBC membrane leads to destruction
- spherocytes and reticulocytes seen on blood film
Clinical features of haemolytic anaemia
Fatigue Malaise Dark urine Acute haemolytic crisis - tachycardia - pallor - jaundice - fever Jaundiced sclera Splenomegaly +- hepatomegaly Gallstones - from bilirubin load
Mx of haemolytic anaemia
Education
- triggers and signs of acute crisis
Folic acid supplementation
Splenectomy
- if spherocytosis causing growth failure
Most post-infectious autoimmune haemolytic anaemia is self-limiting
Haemolysis triggers
Drugs - nitrofurantoin - quinolones - sulphonamides - antimalarials Infections - EBV - mycoplasma Parvovirus B19 Food - fava (broad) beans
Causes of microcytic anaemia
Thalassaemia Anaemia of chronic disease Iron deficiency anaemia Lead poisoning Sideroblastic anaemia
Causes of normocytic anaemia
Acute blood loss Anaemia of chronic disease Aplastic anaemia Haemolytic anaemia Hypothyroidsim
Causes of macrocytic anaemia
Megoblastic - B12 deficiency - folate deficiency Normoblastic - alcohol - reticulocytosis - hypothyroidism - liver disease
