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Yr4 - Paeds > HaemOnc > Flashcards

HaemOnc Flashcards

1
Q

Define leukaemia

A

Malignant disorder of the bone marrow

  • commonest cancer seen within paediatric population
  • most common form is acute lymphoblastic leukaemia (ALL)
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2
Q

Epidemiology of leukaemia

A

Incidence of ALL peaks between 2 and 5 years of age
Males more commonly affected
Genetic diagnoses (trisomy 21) at an increased risk

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3
Q

Pathophysiology of ALL

A

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

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4
Q

Clinical features of ALL

A

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

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5
Q

Ddx of ALL

A

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

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6
Q

Investigations of ALL

A 
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
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7
Q

Risk scoring for ALL

A

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

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8
Q

Mx of ALL

A

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

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9
Q

Prognosis of ALL

A

90% of children survive

Should have regular follow-up and assessment for 5 years then yearly review

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10
Q

Complications of ALL

A

Infertility
Avascular necrosis
Peripheral neuropathy
Anxiety

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11
Q

Define lymphoma

A

Malignancy of the lymphatic system

  • Hodgkin’s
  • non-Hodgkin’s
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12
Q

Epidemiology of lymphoma

A

Over 10% of childhood cancers

  • more commonly seen in boys
  • more common in older children
  • just over half childhood lymphoma are non-Hodgkin
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13
Q

Pathophysiology of lymphoma

A

Multifactorial - infection, genetic and environmental exposures

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14
Q

Risk factors for lymphoma

A

Epstein-Barr virus
Immunosuppression
Other cancers

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15
Q

Clinical features of lymphoma

A 
Visible or palpable mass
- non-tender lymphadenopathy
- mediastinal lymphadenopathy - cough, wheeze, SVCO
History of B symptoms
- weight loss
- nights sweats
- fevers
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16
Q

Differential diagnosis for lymphoma

A 
Reactive lymphadenopathy
- hx of recent infection
Lymphadenitis
- tender and fluctuant lymph nodes
Leukaemia
- lymphadenopathy associated with signs and symptoms of anaemia/thrombocytopenia
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17
Q

Ix for lymphoma

A

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

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18
Q

Risk scoring for lymphoma

A

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

19
Q

Mx of lymphoma

A

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

20
Q

Prognosis of lymphoma

A

Majority of children with recover

- Hodgkin’s lymphoma more favourable prognosis than non-Hodgkin’s

21
Q

Complications of lymphoma

A

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

22
Q

Define nephroblastoma

A

Wilm’s tumour
Most common renal tumour affecting children
Usually occurs unilaterally
- can be bilateral

23
Q

Epidemiology of nephroblastoma

A

80 children per year in the UK
Presents at pre-school age
Slight female predominance

24
Q

Pathophysiology of nephroblastoma

A

Many tumours arise from nephrogenic rests
- embryonal remnant seen in around 1% of infants at birth
Genetics
- mutations of common tumour suppressor genes

25
Q

Risk factors for nephroblastoma

A

Genetic and overgrowth syndromes

  • WAGR - Wilm’s tumour, Aniridia, Genitourinary malformation and Retardation
  • Denys-Drash
  • Beckwith-Wiedemann
26
Q

Clinical features of nephroblastoma

A 
Abdo mass 
- incidental finding or by parents on otherwise well child
Abdo swelling, abdo pain
Fever
Haematuria
27
Q

Ddx for nephroblastoma

A 
Polycystic kidney disease
Hydronephrosis
Neuroblastoma
- abdo mass in pre-school aged child 
- hypertension, abdo pain and fever
- periorbital ecchymosis
28
Q

Ix for nephroblastoma

A

Urine dip - haematuria
Uss
CT/MRI - more detailed and for staging
Biopsy for definitive diagnosis

29
Q

Staging of nephroblastoma

A

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

30
Q

Mx of nephroblastoma

A

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

31
Q

Prognosis of nephroblastoma

A

Good even for metastatic disease

- 85% cured

32
Q

Complications of nephroblastoma

A

Single functioning kidney

Cardiotoxicity due to chemotherapy

33
Q

Risk factors for iron deficiency anaemia

A

Poor dietary intake
Chronic inflammatory conditions
Menorrhagia
Lower socio-economic background

34
Q

Clinical features for iron deficiency anaemia

A 
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
35
Q

Ix for iron deficiency anaemia

A 
Reduced Hb
Reduced MCV
Reduced ferritin
Increased total iron-binding capacity
Reduced transferrin
36
Q

Mx of iron deficiency anaemia

A 
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
37
Q

Causes of haemolytic anaemia

A

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

38
Q

Clinical features of haemolytic anaemia

A 
Fatigue
Malaise
Dark urine
Acute haemolytic crisis
- tachycardia
- pallor
- jaundice
- fever
Jaundiced sclera
Splenomegaly +- hepatomegaly
Gallstones - from bilirubin load
39
Q

Mx of haemolytic anaemia

A

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

40
Q

Haemolysis triggers

A 
Drugs
- nitrofurantoin
- quinolones
- sulphonamides
- antimalarials
Infections
- EBV
- mycoplasma
Parvovirus B19
Food
- fava (broad) beans
41
Q

Causes of microcytic anaemia

A 
Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia
42
Q

Causes of normocytic anaemia

A 
Acute blood loss
Anaemia of chronic disease
Aplastic anaemia
Haemolytic anaemia
Hypothyroidsim
43
Q

Causes of macrocytic anaemia

A 
Megoblastic
- B12 deficiency
- folate deficiency
Normoblastic
- alcohol
- reticulocytosis
- hypothyroidism
- liver disease

Yr4 - Paeds (16 decks)

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