Paediatric Haematology & Malignancy Flashcards Preview

Paediatrics > Paediatric Haematology & Malignancy > Flashcards

Flashcards in Paediatric Haematology & Malignancy Deck (73):
1

Complications of iron deficiency in childhood

Reduced cognitive and psychomotor performance (in the absence of anaemia)

2

CBE, iron studies and blood film in iron deficiency anaemia

Low haemoglobin
Low MCV
Low Ferritin
Low serum iron
Low transferring saturation
High total iron binding capacity
Slightly high platelets
Microcytosis, hypochromia, poikilocytosis, anisocytosis, target cells (severe anaemia), pencil cells, sometimes nucleated RBCs

3

Thalassaemia minor diagnosis

Low MCV (much lower than degree of anaemia)
Diagnosed on Hb electrophoresis (HbA2 greater than 3.5%)
Pre-pregnancy carrier testing of partner is important

4

Macrocytic anaemia in children

Rare in children
Must be investigated and treated urgently if associated with FTT or neurodevelopmental problems

5

Symptoms and signs of anaemia in children

Lethargy
Irritability
Poor feeding
Weakness
Shortness of breath
Pallor
Changes in colour or urine, sclera, skin
+/- splenomegaly
Flow murmur
Signs of cardiac failure

6

Investigations to perform in microcytic anaemia

Iron studies:
- Low ferritin = iron deficiency
- High ferritin = sideroblastic or anaemia of chronic disease
- Normal ferritin - perform electrophoresis

Haemoglobin electrophoresis
HbA2 greater than 3.5%

Lead levels if at risk (chronic lead poisoning also leads to a microcytic anaemia picture)

7

Investigations to perform in undifferentiated anaemia

CBE, blood film and reticulocyte

8

Investigatinos to perform in normocytic anaemia

Reticulocytes:
- increased = haemolysis OR blood loss

Normal reticulocyte count OR abnormalities of other parameters
- hypoplastic/aplastic anaemias (marrow hypoplasias, leukaemia, infiltration)

9

Investigations for haemolytic anaemia

Blood film
- ?RBC abnormalities e.g. spherocytosis
Coomb's test
G6PD screen
Bilirubin
Reticulocytes

10

Management of iron deficiency in children

Dietary modification
- optimise red meat, chicken, green vegetables, fortified foods
- limit cow's milk to less than 500mL/day

Iron supplementation

11

Dosing and duration for iron supplementation in child

2-6mg/kg/day
Split into TDS to reduce gastric irritation
Continue for 3 months to replenish stores

12

Different mixtures of iron supplements for children

Ferrous sulphate
- better absorbed, less tolerated
- e.g. ferro-liquid (6mg.mL elemental iron)
Ferrous gluconate

13

Expected response to adequate iron supplementation in a child

Hb should rise by 10 each week
Follow up with reticulocyte response in 4 weeks

14

Prevention of iron deficiency anaemia in children

Introduction of iron containing solids at 4-6 months
Avoid cow's milk for first 12 months - should only form very minor part of diet up to 24 months
Ensure all formulas and cereals are fortified
Consider supplementation in high risk groups (premmies, low birth weight)

15

Presentation of G6PD deficiency

Acute haemolysis: jaundice, pallor, dark urine
Acute anaemia: faitgue SOBOE or rest, confusion, lethargy

16

How severe must haemolysis be to cause anaemia

Greater than 5% of RBC mass per day

17

Screening and confirmatory tests for G6PD

Fluorescent spot test (most reliable and most sensitive - G6P and NADP to a haemolysate of test RBCs, measure NADPH after by direct fluorescence or nitro blue dye)

Confirmatory:
As above but measures amount of RBC haemolysate, measure NADPH production spectrophotometrically as units per gram of haemoglobin

18

Management of G6PD

Neonatally: manage as other kinds of neonatal jaundice

Acute presentation:
Haemolysis itself is self-limited
Future avoidance of drugs known to trigger haemolysis and fava beans
Transfusion more likely to be required in children and people with comorbidities causing impaired erythropoiesis

19

Diseases indicated by bleeding in joints

Haemophilia A and B

20

Disease processes indicated by mucosal bleeding

Local irritation
Von Willebrane disorder
Platelet dysfunction

21

Disease processes indicated by bleeding of gums, periosteum and skin

Scurvy

22

Disease processes indicated by gastrointestinal bleeding in paediatrics

Haemorrhagic disease of the newborn
Liver disease

23

What is haemorrhagic disease of the newborn?

Vitamin K deficient bleeding in a newborn who has not received a supplemental vitamin K injection

24

Disease processes indicated by retro-orbital bleeding in a child

Haematological malignancy
Disseminated solid tumour

25

Disease indicated by child bleeding from shins only

Not pathological on it's own. Common in pre-schoolers or junior primary children. Ensure to exclude other sites of bleeding

26

Commonest cause of clotting disorders in children

Immune thrombocytopaenic purpura (ITP)

27

Epidemiology of ITP (incidence and common age)

4/100,000 per year
2-10 years, peaks at 5 years

28

Pathophysiology of ITP

Destruction of circulating platelets by anti-platelet IgG autoantibodies causing splenic sequestration

29

Presentation of ITP

Preceding viral infection 1-2 weeks before presentation
Petechiae, purpura and/or superficial bruising
Rarely causes profuse o mucosal bleeding
Bleeding usually occurs abruptly
Examination otherwise normal (nil lymphadenopathy or splenomegaly)

30

Diagnosis of ITP

Diagnosis of exclusion
CBE: platelets usually

31

Management in ITP

Benign and self-limiting in 80%
- Resolves spontaneously in 6-8 weeks
- most children do not require hospitalisation

IF EVIDENCE OF MAJOR HAEMORRHAGE (e.g. intracranial or GI), consider:
- oral prednisolone or IVIG
PLT transfusion reserved for life-threatening haemorrhage (only raises PLT count for few hours)
Advise parents to keep children from playing contact sports while PLT counts very low

32

Complications of ITP

Intracranial bleed (rare)
Chronic ITP
- 10-20% of children who develop ITP
- PLT count remains low 6m after diagnosis
- F>M
- slow insidious onset in children >7y
- screen for development of SLE
Recurrent ITP
- rare
- thrombocytopaenia at over 3 month intervals

33

Prevalence of childhood cancer in Australia

1/500 children below 15y (one of the highest incidences in the world)

34

Most common childhood malignancies

Leukaemia 35% (ALL more)
Primary Brain tumours 20%
Lymphoma 10%
Wilms Tumour 6-8%
Neuroblastoma 6-8%
Rhabdomyosarcoma 5%
Sarcoma of the bone (Osteo- and Ewings) 4%

35

Long term complications of childhood malignancy

Secondary to cancer and to treatments
20x increased risk to general public to develop second cancer
Organ toxicity (heart)
Growth and hormonal deficiency
Infertility
90% of survivors will develop long term effects

36

Genetic syndromes associated with childhood malignancy

Leukaemia:
- trisomy 21***
- Fanconi anaemia
- Neurofibromatosis
- Klinefelter syndrome
- Noonan syndrome
CNS tumours:
- Neurofibromatosis***
- Tuberous sclerosis
Lymphoma:
- Immunodeficiency disorders

37

Presentations of childhood cancer

1. Localised mass
2. Disseminated disease (e.g. bone marrow infiltration)
3. Pressure on local tissue e.g. lymphma - airway obstruction

38

Short term effects of chemotherapy

Bone marrow suppression (anaemia, thrombocytopaenia, neutropenia)
Immunosuppression
Gut mucosal damage -> undernutrition and infection
Nausea and vomiting
Anorexia
Alopecia

39

Long-term side effects of cancer treatment

Renal dysfunction (nephrectomy, cisplatin)
Cardiac dysfunction (doxorubicin, mediastinal radiotherapy)
GH deficiency (Pit RTx)
Bone growth retardation at sites of RTx
Infertility (gonadal RTx, alkylating agents)
Neuropsych (brain surgery or RTx under 5y)
Secondary malignancy (RTx, alkylating agents)
Social/educational disadvantage (absence from school and chronic ill health)

40

Most common types of leukaemia

ALL 4x as common as AML
80% is precursor B cell

41

Survival rates in childhood leukaemia

up to 85%
Less in AML (50-75%)

42

Peak ages of childhood leukaemias

ALL peaks 2-5y
No peak in AML

43

Poor prognostic factors in childhood leukaemia

Age 10
Tumour load >50x10^9
Particular cytogenic/molecular abnormalities
Persistence of leukaemic blasts in marrow after beginning chemotherapy
high submicroscopic levels of leukaemia detected by PCR

44

Treatment of ALL and AML

ALL: CTx - induction, consolidation and CNS directed therapy, re-induction and continuation of maintenance therapy
AML: shorter duration of treatment but more intense with hospitalisation, blood products and lower survival rates

45

What is febrile neutropenia

A single temperature higher than 38.5 OR a sustained temperature higher than 38 for over 1 hour in a patient with confirmed neutropenia or suspected neutropenia (based on CTx in last 14 days)

46

Highest risk patients for febrile neutropenia

AML treatment
Infants with ALL treatment
ALL induction
ALL delayed intensification
Lymphoma induction
Allogenic transplant
Autologous transplant
Re-induction for any chemotherapy

47

Management of febrile neutropenia

On arrival to hospital:
- Check for signs of sepsis
- IV access, fluid resus (20ml/kg bolus), monitoring
- CBE + differential, Biochem, blood culture, group&hold, urine MCS, lactate
- Commence Abx (within 60 minutes, 30min if signs of sepsis)
- Tazocin if not allergic, continue until cultures -ve 48 hours + afebrile 24h
Full assessment after first dose of Abx (full head-to-toe check for any infections) - investigations as indicated

48

What is a neuroblastoma?

A solid extra-cranial tumour of primordial neural crest cells
Common in children

49

Distribution of neuroblastoma

Adrenal gland 40%
Abdominal ganglia 25%
Thoracic ganglia 15%
Cervical ganglia 5%
Pelvic sympathetic ganglia 5%

50

Common presenting symptoms of neuroblastoma

Abdominal mass
Abdominal pain or constipation
Proptosis
Raccoon eyes
Horner syndrome
Localised back pain
Scoliosis
Palpable lymph nodes (if >2y)

51

Investigations in neuroblastoma

CBE: anaemia
Urinary catecholamines: +ve in 90%
Biopsy of mass
Staging: CT/MRI, bone marrow biopsy, bone scan, MIBG scan (90% of neuroblastomas will take up radiolabelled agent)

52

Survival rates in neuroblastoma

approx 30%

53

Managemnt of neuroblastoma

Surgery (localised disease)
Intensive CTx, transplant, radiation, maintenance therapy rescue surgery (metastatic)

54

Syndromes associated with nephroblastoma (Wilm's tumour)

Beckwith-Wiedemann syndrome
Trisomy 18
Deny-Drash syndrome

55

Staging of nephroblastoma (Wilm's tumour)

I-IV
Size (7cm)
Spread from kidneys to vena cava and/or lymph nodes

56

Number of children with nephroblastoma with bilateral diseae on presentation

5%

57

Presentation of nephroblastoma

Painless abdominal swelling
Abdominal pain
Nonspecific symptoms (fever, malaise, anorexia, hypertension, gross haematuria)

58

Characteristic appearance of nephroblastoma on US/CT/MRI

Intrarenal mass
Arising within kidney and enveloping normal renal tissue
Mixed tissue densities (cystic and solid)

59

Cure rate for nephroblastoma

over 90% up to stage III
stage IV: 85-90%

60

Inheritance pattern of haemophilia

X-linked recessive
THUS MUCH MORE COMMON IN MALES

61

Deficiency in haemophilia

Factor VIII in A (80%)
Factor IX in B (20%)

62

Clinical features of haemophilia

Moderate-severe forms:
- bleeding into joints, soft tissues and muscles after minor trauma or spontaneously
- haemarthrosis (pain, local swelling and erythema)
- intracranial haemorrhage (rarely)
Mild disease:
- infrequent bleeding usually secondary to trauma
May only be discovered during routine lab tests or by bleeding after major trauma if 25% normal residual activity

63

Diagnosis of haemophilia

Isolated prolongation of aPTT assay on coagulation studies
Normal platelet counts
Diagnosis made after specific determination of FVIII or FIX clotting activity

64

Management of haemophilia

Factor replacement therapy (acutely or prophylactically)
- FVIII - cryoprecipitate 3days/week
- FXI - 2 days/week
Avoid meds that reduce platelet function (e.g. aspirin)
Antifibrinolytics (EACA or tranexamic acid) to control bleeding in the gums and GI tract

65

Complications of haemophilia

Fatal haemorrhage (oropharyngeal spaces, CNS, retroperitoneum)
Compartment syndrome (secondary to haematoma into muscle in distal limbs)

66

Peak age incidence of paediatric leukaemia

Girls: 2y
Boys: 3y

67

Average treatment time in paediatric leukaemia

Girls: 2y
Boys: 3y

68

Prevention of meningeal disease in leukaemia

intrathecal chemotherapy

69

Good prognostic factors in paediatric ALL

Age 3-7y
Female gender

70

Poor prognostic factors in paediatric ALL

Age less than 1 year or older than 10
WCC greater than 100 at presentation
Philadelphia chromosome

71

Leading cause of death from ITP

intracranial haemorrhage

72

Treatment of choice for a child with ITP with massive haemorrhage

Platelet transfusion
IV methylprednisolone
IVIG
+/- splenectomy

73

Vaccination to cover before undergoing splenectomy

Encapsulated organisms

Pneumococcal
Meningococcal
Haemophilus influenzae B