what happens in ITP
immune. destruction circulating platelets by IgG
what happens in ITP
immune. destruction circulating platelets by IgG
features ITP
2-10y. onset after 1-2w after viral infection. usually short Hx days or weeks.
signs ITP
petechiae, purpura, superficial bruising, epistaxis, other mucosal bleeding. profuse bleeding is uncommon.
what are the platelet levels in ITP
often falls to below 10
diagnosis ITP
of exclusion. younger child- consider congenital eg Bernard soulier syndrome. do bone marrow to exclude acute leukaemia or aplastic anaemia if anaemia, neutropenia, hep splen meg
diagnosis ITP
of exclusion. younger child- consider congenital eg Bernard soulier syndrome. do bone marrow to exclude acute leukaemia or aplastic anaemia if anaemia, neutropenia, hep splen meg
features ITP
2-10y. onset after 1-2w after viral infection. usually short Hx days or weeks.
signs ITP
petechiae, purpura, superficial bruising, epistaxis, other mucosal bleeding. profuse bleeding is uncommon.
what are the platelet levels in ITP
what is a rare complication ITP
intracranial haemorrhage
treatment chronic ITP
supportive. drug treatment only if chronic persistent bleeding affecting daily life, QOL
if going to treat with steroids ITP what should you do
examine bone marrow as could mask ALL
what treatments are there for chronic ITP
ritixumab- antibody against B lymphocytes. thrombopoietic growth factors. splenectomy
when would you treat in ITP
major bleeding or persistent minor bleeding
treatments ITP
oral pred. IV anti D or IVIg. platelet transfusion if life threatening
what is chronic ITP
in 20%, platelet count remains low after 6 months diagnosis
diagnosis neuroblastoma
catecholamine degradation products- VMA, HMMA usually raised
treatment neuroblastoma
chemo with radio and surgery
what treatments are there for chronic ITP
ritixumab- antibody against B lymphocytes. thrombopoietic growth factors. splenectomy
what is a neuroblastoma
malignant tumour arising from sympathetic nervous tissue. derived from neural crest tissue which makes up sympathetic chain and adrenal medulla
where does neuroblastoma commonly develop
adrenal gland
presentation neuroblastoma
abdominal mass, mass can be present anywhere along sympathetic chain. large tumour. early mets to bone, liver, skin.
prognosis Wilms
90% long term survival
treatment neuroblastoma
chemo with radio and surgery
signs retinoblastoma
strabismus (squint) and leukocoria (white pupil). absent red reflex
presentation Wilms tumour
unilateral abdominal mass (95% unilat), often solid and cystic mass. fever, flank pain. haematuria not common.
diagnosis Wilms tumour
US- renal pelvis distortion. hydronephrosis. CT/MRI
management Wilms tumour
avoid biopsy. nephrectomy + vincristine and actinomycin for 4 weeks preop. 2 drugs for early tumour and no radio, 3 drugs for more advanced with radiotherapy
prognosis Wilms
90% long term survival
what is retinoblastoma
most common intraocular tumour in childhood.
signs retinoblastoma
strabismus (squint) and leukocoria (white pupil). absent red reflex
when would you think it is familial (RB)
bilateral. RB1 gene which is a tumour suppressor gene
treatment RB
chemo (good for bilat), enucleation, external beam radiotherapy. screen parents and siblings
what is RB associated with
sarcomas- rhabdosarcoma, osteosarcoma
