haematology and oncology Flashcards
what is iron deficiency anaemia?
iron deficiency anaemia is a global problem affecting 10-30% of those who are high risk and in severe cases can lead to irreversible long term neurodevelopment.
what values define alarm?
<11g/DL under 5s, from 5-11 its under <11.5. and in children older than 12 years old its under 12g/dL
what are the causes of anaemia?
multiple classifications - reduced rbc production, panconis anaemia -bone ,marrow aplasia
bone marrow replacement by tumour cells- leukaemia
replacement by fibrous tissue -granulomas
deficiency of iron or folic acid or vitamin b12
thalasemia
diets low in iron such as vegetarian diets, fad diets
menstruation
growth spurt
gi disorders- such as malabsoprive conditions e.g. coeliac, crowns
risk factors for anaemia?
preterm babies, low body weight and multiple births
what can cause red blood cell destruction
haemolysis, infections
genetics- hereditary spheroctyosis -cell membrane defects
enzyme abnormalities - g6pd
haemoglbinopathies =sickle cell disease, thalassemia
presentation of IDA
fatigue shortness of breath failure to thrive irritability cvs - tachycardia pallor cobjuctiva -pale nail bed palmar creases jaundice-haemolytic
other points to consider in history in IDA
ethnic origin, evidence of blood loss-melaena, haemturia, diet chronic infection family history of conditions recent travel medications
investigations IDA
ferritin, iron, and iron binding capacity
hb
low mcv - thalassemia, iron def or leasd posioning
high mcv- b12, folate deficiency
reticulocyte count increase in anaemia due to immature rbc
folate b12
electrophoresis of haemglobin
blood film
enzyme studies g6pd
coombs test - haemolytic disease
managent IDA
ferrous sulphate supplementation
Sutton for 3 months
transfusion if severe or on verge of cardiac failure/severe illness
address cause
what is wilms tumour?
nephroblastoma with 1 in 20 cases affecting both kidneys
80-85 cases annually in uk
commonest renal cancer in kids
causes of wilms
unknown
may have other genetic abnormalities
symptoms of wills
abdominal mass painless swollen abdo blood in urine increased BP increased renin fever fatigue lack of appetite weight loss
tests for wilms
increased renin
USS
CT MRI
lung mets in 20%
mangement of wilms
nephrotocomy
them and radio therapy
most common type of cancer in kids
leukaemia then brain
which type of brain cancer is most common
astrocytomas (43%) with intra cranial intra spinal
medulloblastoma -20%
what genes have been identified with brain cancer
retinoblastoma -RB1
Neurofibromatosis nf1/2
TUBEROUS SCLERSOSIS-tsc1
clinical features of brain ca
headache which is worsening, worse on walking or lying down vomiting (worse in morning) nausea visual changes- nystagmus, papillodema seizures changes in personality 6th nerve palsy =squint ataxia
failure to thrive
weight loss
investigations for brain tumours
list all!!!
urgent 48 hour referral if suspected ideally mRI but ct if not possible CSF analysis for pineal tumours -AFP Hcg mrI Six monthly excision biopsy but rarely performed
management of brain tumour
presurgery also
types of chemo and radio used
most common ones used
surgical- resection totally best in gliomas
biopsy but hardly achieved
phenytoin to prevent seizures
drain/shunt prevent hydrocephalus
resection below 2y/o vital as radiotherapy not done until later
chemo - vincristine most commonly
radiotherapy- gamma knife, interstitial seeds, localised areas
what is henochs schonlein purpura?
IgA mediated autoimmune hypersensitivity vasculitis
which four organ systems does hSP affect
skin
joints
gut
kidney
who is henochs more common in
20, per 100,000
males
caucasians
4-6 yr old peak pervalence
symptoms of HSP
preceding URTI (often in winter)
low grade fever
symmetrical erythematous rash on ulna side of arm, buttocks, back and evolves into raised purpuric rash looking like rbuises
abdo pain, vomitinh, blood diarrhoea
swollen tender ankles and knees
renal damage- haematuria and proteinuria
headaches
why is renal damage occurring IN HSP
igA deposition in kidney
40% of kids within there months
only 1% go onto ESKD
oliguria and hypertension are rare
investigations in hPS
raised ESR raised creatinine and urea raised white cells dipstick-haemtouria, and proteinuria iGA serum raised autoantibody screen if proteinuria, follow up monthly but if not look at urine dipstick and BP
amangement of HSP
manage proteinuria
self limiting but monitor
Nsaid for joint pains
steroids and aza for renal disease
what can lead to progressive nephritis
plasma exchange
key difference between haemophilia type A and B
clotting factor 8 and 9 respectively
which of the two haemophilia is worse
A
A is 5x more common
haemophilia is inherited how?
x linked recessive
therefore daughters of a father affected will always be a carrier
sons of carrier mother will 50% have it and 50% daughter carries it
symptoms of haemophilia
easy bruising easily bleeds for prolonged time haematuria haemorthrosis haematomas kpomt deformities headache lethargy
investigations of haemophilia
bloods- platelet, prothrombin time normal and vwf
Partial thrombin time is increased
also decrease in factor of relevance
hb reduced if bleeds
which pathways affected haemophilia
intrinsic and common - pthrombin time - A and B
extrinsic and common - partiral prothrombin time
management of haemophilia
replace factors with transfusions
if severe disease , may have antibodies so need to consider desmopressin, tranexamic acid and ffp with factors
also consider ABCDE if acutely unwell
pain relief for joints but nOT NSAIDS nor aspirin(Increased bleeding risk)
what is the most common childhood malignancy?
leukaemia 35%
with 90% being acute lymphoblastic leukaemia alignant disease of the blood-forming organs, characterised by distorted proliferation and development of leukocytes and their precursors in the blood and bone marrow. It can be classified as acute or chronic, according to the degree of cell differentiation (not the duration of disease)
symptoms of leukaemia
SEEek medical help early for persistent signs
I ‘Eye’ signs such as white spot in pupil, squint, blind- ness or bulging eye
L Lumps in abdomen, pelvis, head and neck region, limbs, testes and lymph nodes
U Unexplained fever, LOW, LOA, pallor, fatigue, easy bruising and bleeding
A Aching bones, joints, backache, easy fractures
N Neurological: change in behaviour, balance or gait problems, loss of milestones, headache and increas- ing head circumference
hepatosplenomegaly
testicular enlargement
weakness
night sweats
what are the four types of leukaemia
acute lymphoblastic leukaemia. 78%
chronic lymphoCYTIC leukaemia
acute myeloid leukaemia 15%
chronic myeloid leukaemia -very rare in paediatrics
RF for leukaemia
downs syndrome influenza`a exposure boys causasian ataxia telangiectasia fanconis radiation
investigations for leukaemia
pancytopaenia may elevated wav bone marrow aspiration and biopsy imaging immunophenotyping LP
staging used in leukaemia
franch American British classification used in ALL
what to note regarding immunisation
no routine imms during treatment and 6 months after
