Paeds Haem/Oncology Flashcards
What is the most common paed anaemia?
Iron deficient anaemia
What is the most common cause of iron deficient anaemia?
Inadequate intake
How much iron does a 1yr old need?
8mg/day or 1mg/kg/day
How much iron does a child get from breast milk?
1.5mg/L
How much iron does a child get from cow’s milk?
0.5mg/L
How much iron does a child get from formula milk?
5-9mg/L
What are 2 common causes of inadequate intake of iron?
Delay in starting mixed feeds past 12 months
Low iron foods or lots of cow’s milk in diet
What vitamin increases iron absorption?
Vitamin C
Found in fresh fruit and veg
What decreases the absorption of iron?
Tea (tannin) and high fibre foods (phytates)
When do you start getting symptoms with iron deficient anaemia?
Hb <60-70
What are the symptoms of iron deficient anaemia?
Tiredness, may feed less
Pale - especially tongue and palmar crease
Pica = eating non-food substances eg. soil, chalk, rubber
How is the diagnosis of iron deficient anaemia made?
Blood film = microcytic hypochromic
Low serum ferritin
What are the differentials of iron deficient anaemia? (3)
Beta thalassaemia trait - generally Asian/Arabic kids
Anaemia of chronic disease eg. kidney
Alpha thalassaemia trait - microcytic and hypochromic but not anaemic, African/Far Eastern kids
What is the management of iron deficient anaemia?
Oral Sytron (doesn't stain teeth like other supplements) 3 months
How much will the Hb increase by while taking iron?
10g/L/wk
Why might there be a failure to respond to oral iron?
Coeliac
Blood loss eg. Meckel’s diverticulum
Why is a blood transfusion almost never indicated for iron deficient anaemia?
Because the decline is gradual so they can tolerate Hb as low as 20g/L (similar to COPD with oxygen)
What inheritance does Sickle Cell have?
Autosomal recessive
What is the mutation in SCD?
GAG -> GTG in codon 6 of beta-globin gene
Glutamate -> Valine
What is the main determinant of the severity of SCD?
The amount of HbF vs HbS
Usually have about 1% HbF, but can be up to 15%
Hydroxycarbamide increases HbF production
What exacerbates HbS polymerisation?
Low oxygen
Cold
Dehydration
How much HbS do those with Sickle trait have?
About 40%, asymptomatic
Still transmit to offspring
What is the long-term management of SCD? (4)
Prophylactic Abx due to functional asplenism
Folic acid supplement due to increased RBC turnover
Avoid cold, dehydration, excessive exercise and undue stress - especially careful after swimming and playing outside
Hydroxycarbamide if prone to crises
What is the management of an acute SCD crisis?
Analgesia, fluids, Abx, Oxygen
+/- Exchange transfusion
What are the 3 indications for exchange transfusion?
Acute chest syndrome
Stroke
Priapism
What is acute chest syndrome?
CXR shows bilateral lower consolidation in lungs
What is the management of acute chest syndrome?
CPAP
Exchange transfusion
Abx
When would a bone marrow transplant be offered in SCD?
If very severe crises eg. stroke
90% cure, 5% fatal
What is the life expectancy of SCD?
50% die <40yrs
3% childhood mortality - generally infection
When is SCD tested for?
Heel prick test
Allows early prophylactic Abx
What is HbSC?
A less severe version of SCD, have half HbS, half HbC
What are HbSC patients at risk of?
Proliferative retinopathy - periodic eye checks needed
Osteonecrosis of hips and shoulders in teens
What is beta-thalassaemia major?
No HbA present - need bone marrow transplant if possible
What is beta-thalassaemia intermedia?
Small amount of HbA and large amount of HbF
What is the management of beta-thalassaemia?
Monthly blood transfusions from 3-6 months otherwise fatal
What is the potential side effect of having monthly blood transfusions?
Chronic iron overload
Need chelation from 2-3yrs
How do you chelate chronic iron overload?
Subcut desferrioxamine
Oral desferasirox
Which factors is the PT relevant for?
Factors 2, 5, 7, 10
Which factors is the APTT relevant for?
Factors 2, 5, 8, 9, 10, 11, 12
What does the thrombin time indicate?
Fibrinogen deficieny
What clotting time in relevant for Haemophilias?
APTT
What inheritance is Haemophilia?
X-linked
1/3 is sporadic
What factor is deficient in Haemophilia A?
Factor 8
What factor is deficient in Haemophilia B?
Factor 9
What factors are low in neonates?
All but factor 8 and fibrinogen
What %age of factor 8/9 corresponds with severe, moderate and mild disease?
Severe = <1% - spontaneous muscle/joint bleeds Moderate = 1-5% - bleed after minor trauma Mild = >5% - bleed after major trauma
When does Haemophilia present?
About 1yr, when starting to walk - bruise easily
40% neonatally - IC haemorrhage, bleeding post-circumcision, or continued oozing from heel prick test
What is the ideal management of Haemophilia A and B?
Recombinant factors 8 or 9
Aim to raise to 30% of normal to prevent joint/muscle bleeds
What factor levels are required for surgery?
100% and 50% maintain for 2 weeks following
Need 12hrly/continuous factor infusion
What 3 ‘medications’ need to be avoided in Haemophilia?
All IM injections
Aspirin
NSAIDs
By when is home management encouraged in Heamophilia?
2-3yrs
Child takes over by 7-8yrs
What does severe Haemophilia A require?
Prophylactic factor 8 2-3 times per week
Need to keep above 2%
Why can desmopressin be used to treat mild Haemophilia A?
Desmopressin increases F8:C and vWF production
Ineffective for Haemophilia B
What is a potential complication of have recombinant factors 8/9?
5-20% develop antibodies
Immunosuppression may help
What chromosome is relevant in vWD?
Chromosome 12
What does vWF do?
Allows platelet adhesion to endothelium
Acts as factor 8 carrier protein - prevents factor 8 clearance
What is the inheritance of vWD?
Autosomal dominant
What is the severity of vWD?
Generally mild, presents in teens
What are the symptoms of vWD?
Easy bruising
Prolonged bleeding
Epistaxis
Menorrhagia
Don’t often get soft tissue bleeds or haematomas
What is the management of vWD?
DDAVP (Desmopressin)
Why do you need to be cautious about using desmopressin in children <1yr old?
Can cause water retention and hyponatraemia -> seizures
What is the management of severe vWD?
Plasma-derived Factor 8
DDAVP is inadequate
What is the most common type of leukaemia?
ALL
When does ALL peak?
2-5 years old
What a the 3 main features of ALL?
Anaemia
Neutropenia
Thrombocytopenia
What may be found on examination of someone with ALL?
Bruises
Hepatosplenomegaly
Enlarged LNs
What type of cells may be seen on a blood film in ALL?
Blast cells
What is required for a diagnosis of ALL?
Bone marrow biopsy
What other investigations (not bloods) are useful in ALL?
LP - checks if blast cells in CSF
CXR - checks for mediastinal LNs
What 3 things suggest a worse prognosis?
Age <1 or >10yrs
WCC >50
Blast cells in marrow
What is the management of ALL?
Chemotherapy
Blood and platelet transfusion
Allopurinol and hydration protects kidneys
What is the chemotherapy regime for ALL?
Induction of remission
Intensification to consolidate remission - includes intrathecal chemo to prevent CNS relapse
Continuation - 3yrs of moderate chemo
What must you give alongside chemo to prevent Pneumocystis in ALL?
Clotrimazole prophylaxis
When does Hodgkin and NHL present?
HL = teens NHL = children
What is lymphoma?
Malignancy of immune cells
How does Hodgkin lymphoma present?
Painless firm lymphadenopathy for a long time - may cause airway obstruction
B-symptoms uncommon
What virus is HL associated with?
EBV - 50% of cases
What type of cells may be seen on a blood film of HL?
Reed-Sternberg cells
What is the management of HL?
Chemo-radiotherapy
80% cure rate
What is the presentation of NHL?
Mediastinal mass if T-cell disease - may cause SVCO
Enlarged LNs if B-cell disease
Abdo pain if causing intestinal obstruction
What is the management of NHL?
Multiagent chemo
80% cure rate
What is Burkitt’s Lymphoma?
Type of NHL
c-myc gene
What are the 3 variants of Burkitt’s Lymphoma?
Endemic variant
Sporadic variant
Immunodeficiency variant
What is the endemic variant?
Common in Africa
Almost all have EBV
Involves facial and jaw bones
What is a neuroblastoma?
Tumour from neural crest tissue in adrenal medulla and sympathetic nervous system
When do neuroblastoma occur?
> 5yrs
Occasionally young infants - regress spontaneously
What are the symptoms of a neuroblastoma?
Large abdominal mass from adrenal glands, often crosses midline and involves major vessels
Mets -> bone pain, BM suppression, weight loss
What 3 investigations should be performed for neuroblastoma?
Urinary catecholamine metabolites (VMA, VHA) - increased
BM biopsy
MIBG scan for mets
Why is the prognosis often poor for neuroblastoma?
Often present >1yr with mets
What is the cure rate for neuroblastoma?
40%
What is the management of neuroblastoma?
Single lesion = surgery
Mets = high-dose chemo with stem cell rescue
When do Wilm’s tumour present?
80% <5yrs
What is the usual presentation of Wilm’s tumour?
Large abdominal mass found incidentally
Occasionally haematuria
What investigations should you perform for Wilm’s tumour?
USS +/- CT/MRI
Shows intrinsic renal mass
What is the management of Wilm’s tumour?
Chemo followed by delayed nephrectomy
What is the cure rate for Wilm’s tumour?
80%
60% if mets
What is the most common paediatric brain tumour?
Astrocytoma, 40%
What is a particularly aggressive form of astrocytoma?
Glioblastoma Multiforme
Where would a medulloblastoma be found?
In the midline of the posterior fossa
Why do 20% of patients with medulloblastoma have spinal mets?
Spreads through CSF
Where would a ependymoma be found?
Posterior fossa - similar to medulloblastoma
How common are brainstem gliomas?
4%, very poor prognosis
Where are craniopharyngomas found?
Suprasellar region
Grow from remnant of Rathke pouch
What visual disturbance can craniopharyngomas cause?
Bitemporal hetronymous hemianopia
Where are astrocytomas often found?
Frontal lobe
14yr old with aggressive behaviour, seizures and headache
Astrocytoma
<30% survival
10yr old with headache, vomiting, poor growth, visual changes and diabetes insipidus
Craniopharyngoma
Good survival rate but long-term vision and pituitary issues
3yr old with morning vomiting, unsteady on feet and new squint
Medulloblastoma
50% 5yr survival
4yr old refusing to walk with squint, facial asymmetry and drooling
Brainstem glioma
<10% survival - palliative treatment
What investigations do you need for brain tumours?
CT head then MRI
LP - contraindicated in increase ICP
What is the management of brain tumours?
Surgery - reduce hydrocephalus and attempt maximal resection, not with brainstem tumours
RTx/chemo depends on tumour type
