haem/ oncology

Question 1

what happens in ITP

Answer 1

immune. destruction circulating platelets by IgG

Question 2

what happens in ITP

Answer 2

immune. destruction circulating platelets by IgG

Question 3

features ITP

Answer 3

2-10y. onset after 1-2w after viral infection. usually short Hx days or weeks.

Question 4

signs ITP

Answer 4

petechiae, purpura, superficial bruising, epistaxis, other mucosal bleeding. profuse bleeding is uncommon.

Question 5

what are the platelet levels in ITP

Answer 5

often falls to below 10

Question 6

diagnosis ITP

Answer 6

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

Question 7

diagnosis ITP

Answer 7

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

Question 8

features ITP

Answer 8

2-10y. onset after 1-2w after viral infection. usually short Hx days or weeks.

Question 9

signs ITP

Answer 9

petechiae, purpura, superficial bruising, epistaxis, other mucosal bleeding. profuse bleeding is uncommon.

Question 10

what are the platelet levels in ITP

Question 11

what is a rare complication ITP

Answer 11

intracranial haemorrhage

Question 12

treatment chronic ITP

Answer 12

supportive. drug treatment only if chronic persistent bleeding affecting daily life, QOL

Question 13

if going to treat with steroids ITP what should you do

Answer 13

examine bone marrow as could mask ALL

Question 14

what treatments are there for chronic ITP

Answer 14

ritixumab- antibody against B lymphocytes. thrombopoietic growth factors. splenectomy

Question 15

when would you treat in ITP

Answer 15

major bleeding or persistent minor bleeding

Question 16

treatments ITP

Answer 16

oral pred. IV anti D or IVIg. platelet transfusion if life threatening

Question 17

what is chronic ITP

Answer 17

in 20%, platelet count remains low after 6 months diagnosis

Question 18

diagnosis neuroblastoma

Answer 18

catecholamine degradation products- VMA, HMMA usually raised

Question 19

treatment neuroblastoma

Answer 19

chemo with radio and surgery

Question 20

what treatments are there for chronic ITP

Answer 20

ritixumab- antibody against B lymphocytes. thrombopoietic growth factors. splenectomy

Question 21

what is a neuroblastoma

Answer 21

malignant tumour arising from sympathetic nervous tissue. derived from neural crest tissue which makes up sympathetic chain and adrenal medulla

Question 22

where does neuroblastoma commonly develop

Answer 22

adrenal gland

Question 23

presentation neuroblastoma

Answer 23

abdominal mass, mass can be present anywhere along sympathetic chain. large tumour. early mets to bone, liver, skin.

Question 24

prognosis Wilms

Answer 24

90% long term survival

Question 25

treatment neuroblastoma

Answer 25

chemo with radio and surgery

Question 26

signs retinoblastoma

Answer 26

strabismus (squint) and leukocoria (white pupil). absent red reflex

Question 27

presentation Wilms tumour

Answer 27

unilateral abdominal mass (95% unilat), often solid and cystic mass. fever, flank pain. haematuria not common.

Question 28

diagnosis Wilms tumour

Answer 28

US- renal pelvis distortion. hydronephrosis. CT/MRI

Question 29

management Wilms tumour

Answer 29

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

Question 30

prognosis Wilms

Answer 30

90% long term survival

Question 31

what is retinoblastoma

Answer 31

most common intraocular tumour in childhood.

Question 32

signs retinoblastoma

Answer 32

strabismus (squint) and leukocoria (white pupil). absent red reflex

Question 33

when would you think it is familial (RB)

Answer 33

bilateral. RB1 gene which is a tumour suppressor gene

Question 34

treatment RB

Answer 34

chemo (good for bilat), enucleation, external beam radiotherapy. screen parents and siblings

Question 35

what is RB associated with

Answer 35

sarcomas- rhabdosarcoma, osteosarcoma