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Paediatric Haematology & Malignancy Flashcards

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1
Q

Complications of iron deficiency in childhood

A

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

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2
Q

CBE, iron studies and blood film in iron deficiency anaemia

A 
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
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3
Q

Thalassaemia minor diagnosis

A 
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
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4
Q

Macrocytic anaemia in children

A

Rare in children

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

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5
Q

Symptoms and signs of anaemia in children

A 
Lethargy
Irritability
Poor feeding
Weakness
Shortness of breath
Pallor
Changes in colour or urine, sclera, skin
\+/- splenomegaly
Flow murmur
Signs of cardiac failure
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6
Q

Investigations to perform in microcytic anaemia

A

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)

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7
Q

Investigations to perform in undifferentiated anaemia

A

CBE, blood film and reticulocyte

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8
Q

Investigatinos to perform in normocytic anaemia

A

Reticulocytes:
- increased = haemolysis OR blood loss

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

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9
Q

Investigations for haemolytic anaemia

A 
Blood film
 - ?RBC abnormalities e.g. spherocytosis
Coomb's test
G6PD screen
Bilirubin
Reticulocytes
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10
Q

Management of iron deficiency in children

A

Dietary modification

  • optimise red meat, chicken, green vegetables, fortified foods
  • limit cow’s milk to less than 500mL/day

Iron supplementation

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11
Q

Dosing and duration for iron supplementation in child

A

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

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12
Q

Different mixtures of iron supplements for children

A

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

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13
Q

Expected response to adequate iron supplementation in a child

A

Hb should rise by 10 each week

Follow up with reticulocyte response in 4 weeks

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14
Q

Prevention of iron deficiency anaemia in children

A

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)

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15
Q

Presentation of G6PD deficiency

A

Acute haemolysis: jaundice, pallor, dark urine

Acute anaemia: faitgue SOBOE or rest, confusion, lethargy

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16
Q

How severe must haemolysis be to cause anaemia

A

Greater than 5% of RBC mass per day

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17
Q

Screening and confirmatory tests for G6PD

A

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

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18
Q

Management of G6PD

A

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

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19
Q

Diseases indicated by bleeding in joints

A

Haemophilia A and B

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20
Q

Disease processes indicated by mucosal bleeding

A

Local irritation
Von Willebrane disorder
Platelet dysfunction

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21
Q

Disease processes indicated by bleeding of gums, periosteum and skin

A

Scurvy

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22
Q

Disease processes indicated by gastrointestinal bleeding in paediatrics

A

Haemorrhagic disease of the newborn

Liver disease

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23
Q

What is haemorrhagic disease of the newborn?

A

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

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24
Q

Disease processes indicated by retro-orbital bleeding in a child

A

Haematological malignancy

Disseminated solid tumour

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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

Paediatrics (20 decks)

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