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Paediatrics > Oncology & Haematology > Flashcards

Oncology & Haematology Flashcards

1
Q

What are the age incidences in cancers?

A

ALL - 2-6yo
Non-Hodgkin lymphoma - childhood
Hodgkin lymphoma - adolescence

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2
Q

What is acute lymphoblastic leukaemia?

A

Excessive proliferation of lymphocytes –> many immature lymphocytes
Presents in 2-6yo
Presents insidiously over several weeks

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3
Q

How does ALL present?

A 
Malaise and anorexia
Bone marrow infiltration
- anaemia (lethargy)
- neutropenia (infection)
- thrombocytopenia (bruising, petechiae, nose bleeds)
- bone pain
Reticule-endothelial infiltration
- hepatosplenomegaly
- lymphadenopathy
Other organs
- CNS (nerve pansies, headache, vomiting)
- testes (enlargement)
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4
Q

How is ALL investigated?

A

FBC - low haemolytic, thrombocytopenia, circulating leukaemic blast cells
Bone marrow - indicates prognosis
CXR - mediastinal mass

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5
Q

How is ALL treated?

A

0-4 weeks - induction

  • steroids
  • IV vincristine
  • IT methotrexate
  • L-asparaginase

5-8 weeks - consolidation

  • steroid
  • vincristine
  • IT methotrexate
  • thiopurine

Maintenance treatment (2yrs girls, 3yrs boys)

  • methotrexate (oral and IT)
  • prophylactic co-trimoxazole (prevent pneumonia)
  • vincristine

Blood transfusions (platelets and whole red cells) - reduce symptoms

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6
Q

What is tumour lysis syndrome?

A

Systemic and rapid release of intracellular contents as chemotherapy destroys blast cells

  • Increased urea
  • Increased phosphate
  • Increased potassium
  • Decreased calcium

Can give allopurinol

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7
Q

What are negative prognostic factors for ALL?

A

10yo
>50 WBC
Male gender
CNS involvement

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8
Q

What is lymphoma?

A

Proliferation of cells in lymphatic system (nodes, spleen and liver)

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9
Q

How does non-Hodgkin’s lymphoma present, how is it investigated and treated?

A

Affects lymph nodes and younger children

Lymphoblastic (mainly T-cell)

  • anterior mediastinal mass
  • bone marrow, bone, skin, CNS, liver, kidneys, spleen

B cell malignancy (Burkitt)
- lymph nodes in head, neck or abdomen –> pain, intussusception

Ix: bone marrow aspirate, LP, CT, PET scan
Tx: chemotherapy
Px: >80%

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10
Q

How does Hodgkin’s lymphoma present, how is it investigated and treated?

A

Previous EBV infection, adolescents
Reed-Sternberg cells

Painless lymphadenopathy (neck or mediastinal)

Ix: CT neck, chest, abdo and pelvis, PET scan, bone marrow aspirate, EBV serology
Tx: radiotherapy +/- chemo
Px: >90%

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11
Q

How does NHL and HL differ?

A

HL

  • Reed-Sternburg cells
  • upper body
  • spreads slowly and receptive to Tx
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12
Q

What are the side effects of chemotherapy?

A 
Hair loss
Anaemia
Infection
Bruising
Sore mouth
N+V
Weight gain
Delayed puberty
Reduced fertility and growth
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13
Q

What is neuroblastoma?

A

Arise from neural crest tissue in adrenal medulla and sympathetic nervous tissue

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14
Q

What are the most common cancers in children?

A 
Leukaemia
CNS tumours
Lymphoma
Neuroblastoma
Soft tissue sarcoma
Wilms tumour
Bone tumour
Retinoblastoma
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15
Q

Where does haemopoiesis occur?

A

Fetal - liver

Postnatal - bone marrow

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16
Q

What are the physiological changes in blood count from neonate to adolescence?

A

Hb - starts high, falls due to increased RBC production and then levels off at adult levels

WBC - starts high and decreases

Platelet - similar to adult

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17
Q

How does iron deficiency arise and how does it present?

A

Common - sourced from breast milk, formula, cow’s milk or solids
May be due to delay in weaning

Child will tire easily and feed slowly

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18
Q

What does folate deficiency cause?

A

Body can’t make enough RBC so macrocytic megaloblastic anaemia

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19
Q

What is thrombocytopenia and what does it cause?

A

Platelet count bruising, petechiae, purpura, mucosal bleeding

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20
Q

What is immune thrombocytopenia?

A

Destruction of platelets by anti-platelet IgG autoantibodies

Usually post URTI

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21
Q

How does ITP present?

A

1-2 weeks post URTI
Petechiae, purpura or superficial bruising
Epistaxis, profuse and intracranial bleeding is rare

Mostly acute and self-limiting

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22
Q

How is ITP treated?

A

Oral prednisolone, IV anti-D or IVIG

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23
Q

What is Von Willebrand disease?

A

Deficiency in factor responsible for platelet adhesion and carrying factor VIII

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24
Q

How does pattern of bleeding help differentiate cause?

A

Into mucous membranes and skin - platelet disorder or vWD

Into muscles or joints - haemophilia

Scarring and delayed haemorrhage - disorders of connective tissue

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25
Q

What is haemolytic anaemia?

A

Reduced RBC lifespan due to increased intra and extravacular destruction
Leads to hepatosplenomegaly, increased unconjugated bilirubin and urinary urobilinogen

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26
Q

What are the causes of haemolysis?

A

Neonates - autoimmune

Childhood - hereditary spherocytosis, G6PD deficiency, PK deficiency, thalessaemia, sickle cell disease

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27
Q

What is hereditary spherocytosis?

A

Mutation in gene encoding RBC skeletal protein

RBC loses some of membrane going through spleen –> spheroidal and is destroyed

28
Q

How does hereditary spherocytosis present and how is it treated?

A

Around birth
Jaundice, anaemia, splenomegaly, gallstones

Folate supplementation or splenectomy

29
Q

What is G6PD deficiency?

A

Commonest RBC problem worldwide esp. central Aftrica

X-linked but female carriers are clinically normal

30
Q

How does G6PD deficiency present?

A

Neonatal jaundice

Acute haemolysis precipitated by infection, drugs, ?broad beans

31
Q

What is pyruvate kinase deficiency?

A

Lack of enzyme which decreases ATP

Cell becomes rigid and is destroyed in spleen

32
Q

What is haemophilia A?

A

Factor VIII deficiency

More common

33
Q

What is haemophilia B?

A

Factor IX deficiency

34
Q

How does haemophilia present?

A

Most present by 1yo when walking starts
Recurrent spontaneous bleeding into joints and muscles –> arthritis

In neonates - intracranial haemorrahge, oozing from heel prick or post circumcision

35
Q

What do neuroblastoma arise from?

A

Neural crest tissue in adrenal medulla ad sympathetic nervous tissue

36
Q

What is sickle cell disease?

A

Haemoglobinopathies in which HbS mutations are inherited

Change in amino acid from glutamine to valine

37
Q

What is sickle cell anaemia?

A

HbSS (homozygous)

All Hb is HbS (small amount of fetal Hb)

38
Q

What is sickle-cell haemoglobin C disease?

A

HbS and HbC from each parent
HbC is different mutation in B-globlin, glutamine to lysine
Not as severe as sickle cell anaemia (HbC doesn’t polymerise as quickly)

39
Q

What is sickle B-thalassaemia?

A

HbS and B-thalassaemia

No HbA so similar symptoms to sickle cell anaemia

40
Q

What is sickle trait?

A

Inherit HbS and normal B-globin gene

Carriers and asymptomatic

41
Q

What does HbS do?

A

Polymerises within RBC to form rigid tubular spiral bodies which deform cells into sickle cells
Reduced life span and may be trapped in micro-circulation –> blood vessel occlusion and ischaemia
Exacerbated by low oxygen tension, dehydration and cold

42
Q

How does sickle cell disease present?

A

Anaemia - clinically detectable jaundice

Infection - increased susceptibility to pneumococci and H. influenzae (hyposplenism secondary to microinfarction)

Painful crises - vaso-occlusion in hands and feet, chest

Splenomegaly

Priapism (erectile dysfunction)

43
Q

What are the long term complications of sickle cell disease?

A 
Short stature and delayed puberty
Stroke and cognitive problems
Adeno-tonsillar hypertrophy
Cardiac enlargement and heart failure - chronic anaemia
Renal dysfunction
Pigmented gallstones
44
Q

How is sickle cell disease managed?

A

Prophylaxis - full immunisation and daily oral penicillin
Daily folic acid
Avoid vaso-occlusive crises - analgesia and hydration if occur

45
Q

How is sickle cell disease screened for?

A

Guthrie test

Chorionic villus sampling

46
Q

What is B-thalassaemia and what are the different types?

A

Occur in people from Indian subcontinent, Mediterranean and Middle East

Reduction of production of B-globin –> reduction in HbA

B-thalassaemia major - most severe, HbA cannot be produced

B-thalassaemia intermedia - milder, B-globin mutations allow small amount of HbA to be produced

47
Q

How does B-thalassaemia present?

A

Severe anaemia and jaundice
Failure to thrive
Extramedullary haemopoiesis –> hepatosplenomegaly and bone marrow expansion (maxillary overgrowth and skull bossing)

48
Q

What haematological disorder causes a change in facial structure?

A

B-thalassaemia

Bone marrow expansion in maxillary

49
Q

How is B-thalassaemia managed?

A

Lifelong monthly blood transfusions - keep Hb above 100g/l to reduce growth failure and bone deformation

This can cause chronic iron overload –> cardiac failure, liver cirrhosis, diabetes, infertility, growth failure
Treated with iron chelation from 2 years old

Bone marrow transplant will cure

50
Q

What is the B-thalassaemia trait?

A

Heterozygotes are asymptomatic

RBC are hypochromic and microcytic

51
Q

What is alpha-thalassaemia?

A

Healthy people have 4 alpha-globin genes

No alpha-globin = alpha-thalassaemia major, fetal hydrops

1 alpha-globin = mild to moderate anaemia

2 or 3 alpha-globin = asymptomatic

52
Q

What is a Wilm’s tumour?

A

Nephroblastoma - originates from embryonic renal tissue

Most present before 5 years old and it’s very rare after 10

Associated with trisomy 18

53
Q

How does a Wilm’s tumour present?

A

Large abdominal mass in otherwise well child

Usually metastasises to lung

54
Q

How is Wilm’s tumour treated?

A

Chemo plus delayed nephrectomy

Prognosis is good >80% but recurrence has poor prognosis

55
Q

What is rhabdomyosarcoma?

A

Soft tissue sarcoma

Originates from primitive mesenchymal tissue - head and neck, GU

56
Q

How does rhabdomyosarcoma present?

A

Head and neck - proptosis, nasal obstruction or bloody nasal discharge

GU (bladder, paratesticular or urethra) - dysuria, urinary obstruction, blood stained vaginal discharge

57
Q

What is the prognosis for rhabdomyosarcoma?

A

Metastasis is present in 15% and associated with poor prognosis

58
Q

When and how do bone tumours present?

A

Uncommon before puberty
Osteosarcomas are more common
Ewings seen more often in younger children

Persistant bone pain in otherwise well patient

59
Q

How are bone tumours diagnosed and treated?

A

X-ray - destruction and new bone formation, Ewing’s has soft tissue mass

Chest CT - lung metastases

Bone marrow sample - exclude marrow involvement

60
Q

What is disseminated intravascular coagulation?

A

Disorder of coag pathway –> diffuse fibrin deposition in microvasculature and consumption of coag factors and platelets

Tiny clots and severe bleeding

61
Q

What are the causes of DIC and how does it present?

A

SEPSIS or SHOCK
Meningococcal septicaemia
Trauma and burns

Bruising, purpura, haemorrhage

Thrombocytopenia, prolonged prothrombin or APTT, low fibrinogen

62
Q

How do retinoblastoma present?

A

Malignant tumour of retinal cells, may affect one or both (hereditary) eyes

Most present within 3 years

White pupillary reflex replaces red one

63
Q

How are retinoblastomas treated?

A

Cure but preserve vision if possible

Chemotherapy to shrink tumour and then local laser treatment

64
Q

What are the indications for splenectomy?

A

Hereditary spherocytosis, lymphoma, ITP

Trauma

65
Q

What are the complications of splenectomy?

A

Low dose Abx needed for life

Malaria

66
Q

What are side effects of chemotherapy?

A

Bone marrow suppression - anaemia, thrombocytopenia, bleeding
Immunosuppression - infection
Gut mucosal damage - infection and undernutrition
N+V, anorexia - undernutrition
Alopecia

Paediatrics (14 decks)

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