Paed Haem/Onc Flashcards
Distribution of major childhood cancers
-Leukaemia 34%
-Brain/ spinal 24%
-Embryonal 15%
-Lymphoma 11%
-Soft tissue 6%
-Bone 5%
-Other 5%
Overview of Idiopathic Thrombocytopenia Purpura
-Immune disease affecting platelets, causing petechaie
-Check a full blood count in children with a non-blanching rash, refer to paediatrician
-ITP = non-blanching rash due to low platelets
-Consider non-accidental injury in any child with unexplained bruising.
-Immune (or idiopathic) thrombocytopenic purpura (ITP) is an immune-mediated reduction in the platelet count. Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex. It is an example of a type II hypersensitivity reaction.
ITP in children is typically more acute than in adults and may follow an infection or vaccination.
Features
bruising
petechial or purpuric rash
bleeding is less common and typically presents as epistaxis or gingival bleeding
Investigation
full blood count
should demonstrate an isolated thrombocytopenia
blood film
bone marrow examinations is only required if there are atypical features e.g.
lymph node enlargement/splenomegaly, high/low white cells
failure to resolve/respond to treatment
Management
usually, no treatment is required
ITP resolves in around 80% of children with 6 months, with or without treatment
advice to avoid activities that may result in trauma (e.g. team sports)
other options may be indicated if the platelet count is very low (e.g. < 10 * 109/L) or there is significant bleeding. Options include:
oral/IV corticosteroid
IV immunoglobulins
platelet transfusions can be used in an emergency (e.g. active bleeding) but are only a temporary measure as they are soon destroyed by the circulating antibodies
Overview of acute lymphoblastic leukaemia
-Most common malignancy affecting children and accounts for 80% of childhood leukaemias. The peak incidence is at around 2-5 years of age and boys are affected slightly more commonly than girls
-Peak age 2-4, 85% ALL, 10% AML, rest CML/MDS
-P: anaemia (lethargy and pallor), neutropaenia (frequent or severe infections), thrombocytopenia (easy bruising, petechiae), bone pain (secondary to bone marrow infiltration), splenomegaly, hepatomegaly, lymphadenopathy (axillary/ inguinal), SVC obstruction, fever is present in up to 50% of new cases (representing infection or constitutional symptom), testicular swelling, misery, fatigue
-Types: common ALL (75%), CD10 present, pre-B phenotype, T-cell ALL (20%), B-cell ALL (5%)
-I: Check a full blood count in children with a non-blanching rash, differential white count including blast count, urate tumour burden), CXR (child with acute leukaemia has XR for general anaesthesia and central venous line, as mediastinal mass common affecting airway), biochemistry esp renal function, Consider non-accidental injury in any child with unexplained bruising
-Poor prognostic factors:
=Age < 2 years or > 10 years
=WBC > 20 * 109/l at diagnosis
=T or B cell surface markers
=Non-Caucasian
=Male sex
-Good and poor cytogenetics, minimal residual disease (MRD) very important diagnosis + day 29: different arms of treatment protocol. Treat sanctuary sites (testes in boys, CNS: spinal cord intrathecal chemotherapy via lumbar puncture), good prognosis
-B cell precursor: WCC <50 x 10^9/L + age 1- years: NCI standard risk (A- 3 drug induction) vs 10+ >50 4 drugs
-T cell 4 drugs
-IV hyperhydration + allopurinol or rasburicase (dissolve uric acid to prevent tumour lysis), blood + platelet Tx, BMA, LP and intrathecal methotrexate, Vincristine good for leukaemia), steroids, asparaginase
Overview of Neuroblastoma
-Second most common solid tumour in HIC, ost common outside CNS, 10% all paediatric cancers but 50% neonatal tumours. M:F 1.2:1, 15% of childhood cancer deaths, majority at stage 4 at presentation due to tumour genetics), median age of diagnosis 18 months, vast majority <5 years old, rarely >10 years
-Primary sites: pain, abdo distention, hepatomegaly, visual/palpable mass, resp distress, urinary retention, constipation. Bone pain and limp, pallor, weight loss. Neck/chest/paraspinal (20%): resp distress, cord compression. Pressure of nerves, paraplegia, proptosis
-P: hypertension (catecholamine secretion renal artery compression, urinary tract obstruction), Horner’s syndrome: interruption of sympathetic nerves supplying face (ptosis miosis, anhidrosis). Mets (bone marrow, bone: limp/ refusal to walk/pain and irritability/proptosis/per orbital ecchymosis, blueberry muffin spots, opsoclonus/ myoclonus: dancing eyes and feet/ paraneoplastic inflammatory neurologic disorder/ chaotic limb and eye movements/ ataxia/ irritability and behavioural changes, anorexia/weight loss)
Sick child and can mimic acute leukaemia
-I: history and exam, blood tests, urine (raised urinary VMA and HVA levels), imaging, tissue diagnosis, staging, calcification on AXR, biopsy
Overview of Wilms tumour
-Well child with coincidental finding of abdominal mass. Wilms’ tumour is a specific type of tumour affecting the kidney in children, typically under the age of 5 years.
-Differentials: chronic myeloid leukaemia, neuroblastoma
-P: abdo pain, haematuria, lethargy, fever, hypertension, weight loss
-USS: no calcification, does not cross midline, check mets in liver, growth in IVC? in MRI
-Good prognosis (almost everyone cured), biopsy or not (not if radiologically typical, no other features), chemotherapy (post op)+ surgery (nephrectomy), radiotherapy occasionally. Predisposition syndromes (Beck: hypoglycaemia)
Symptoms of brain tumours
-Headache
-Vomiting
-Ataxia
-Focal / generalized seizures
-Visual pathway problems
-Failure to thrive
-Irritability
-Growth and endocrine issues
-Behaviour problems
-Papilledema
Investigation of brain tumour
-MRI
-Not CT as radiation risk for children
-Differentials: tumour, cerebral trauma, haemorrhage
Management of brain tumour
-Semen cryopreservation
-Central lines
