Haematology and Oncology Flashcards
ITP + AIHA =
Evan’s syndrome
Which immunoglobulin drives ITP?
IgG
3 features of craniopharyngioma
headache
visual disturbance
hormone imbalance
G6PD blood results
Low - Hb, haptoglobin, spectrophotometric assay <10%
High - reticulocytes, bili, lactate
Present - Heinz cells, bite cells, polychromasia
Factor 8 levels in severe haemophilia A
<1%
most important treatment in sickle cell
high flow O2 (hypoxia drives sickling)
What do Howell Jolly bodies show?
splenic dysfunction e.g. sickle cell
onset of high grade astrocytoma
acute
sickle cell diagnosis
electrophoresis
how does sickle cell cause macrocytosis?
chronic haemolysis = folate deficiency
alpha thalassaemia deletions
1 or 2 = trait
3 = HbH disease
4 = foetal hydrops and fatal Bart’s
beta thalassaemia mutations
1 = minor
2 = major
most common solid tumour in children and location
medulloblastoma (cerebellar)
how to confirm aplastic crisis
reticulocytes inappropriately low
who gets Hodgkin’s lymphoma?
teenagers
G6PD inheritance
X linked recessive
hereditary spherocytosis inheritance
AD
sickle cell and thalassaemia skull deformities
sickle cell = overbite
thalassaemia = bossing
haemophilia coag results
raised aPTT
normal bleeding time
supraclavicular LN enlargement?
do CXR for ?mediastinal involement ?cancer
tuberous sclerosis presentation
epilepsy
macules
shagreen patch
fibroma/lipoma/harmatomas
dental pits
renal cysts
regular Ix for tuberous sclerosis
MRI head and abdo
haptoglobin in haemolysis
low
ependyoma location
extends into the upper cervical cord through the foramen magnum
test for neuroblastoma
urine catecholamines
bloods in hereditary spherocytosis
anaemia
unconjugated jaundice
major haemorrhage management
20ml/kg RBC
if still shocked
10ml/kg cryoprecipitate
most common childhood leukaemia
ALL
first treatment in ALL
correct anaemia and platelets before chemo
timing of CNS infiltration in ALL
relapse rather than diagnosis
Fanconi’s anaemia inheritance
autosomal recessive
drug to avoid in haemaphilia
NSAIDs
which haemophilia may be managed by desmopressin if mild?
A
iron content in milk
Cows milk is higher than breast but more poorly absorbed
which iron preparations for children and why
edentate or polysaccharide (others stain teeth)
test before steroids for ITP
bone marrow biopsy to exclude ALL
when to give a platelet transfusion
life threatening bleed (only raises plt for a few hours)
G6PD activity in crisis
high
when to do splenectomy in hereditary spherocytosis
> 7yo due to sepsis risk
2 conditions associated with spherocytes and how to differentiate
hereditary spherocytosis
AIHA
do DAT
LT drug for sickle cell
hydroxycarbamide
post stroke management of sickle cell
sibling bone marrow transplant
Rx for mild von willebrand
desmopressin
Rx for severe von willebrand
plasma derived factor 8 concentrate
Which AIHA is more common
warm (IgG)
how does warm AIHA cause haemolysis
extravascular
AIHA presentation
dark urine
anaemia
splenomegaly
DVT
where does foetal haematopoesis occur
liver
Fetomaternal alloimmune thrombocytopaenia
Pathophysiology
Presentation
Treatment
Baby inherits dad’s platelets with antigens on. Sensitisation event occurs and mum develops attacking Ab that can cross the placenta and attack babies platelets. (Mum attacks baby).
Low Plts = purpura and intracranial haemorrhage in otherwise well baby and mum
Give antigen negative platelets (irradiated and plasma removed)
DAT in hereditary spherocytosis
negative
when to do a bone marrow biopsy in ITP
bone pain or limp
abnormal lymphadenopathy
hepatosplenomegaly
persistent fever
macrocytosis
FH or PMH of bleeding
other abnormalities on FBC
DIC bloods
low fibrinogen
high PT
how to differentiate AML and ALL
myeloperoxidase is positive in AML
