Oncology and haematology Flashcards
In what ethnicity is iron deficiency anaemia in children most common?
Those from the Indian subcontinent
What is the daily iron requirement for children and toddlers?
High - 1mg/kg/day
How is iron deficiency anaemia diagnosed in children?
Hypochromic microcytic anaemia + low serum ferritin
How is iron deficiency anaemia managed in children?
Dietary advice + oral iron replacement therapy for 3/12
What are the broad causes of anaemia?
- Decreased/difficulty in red cell production
- Increased red cell destruction
- Combination of causes, e.g. anaemia of prematuirty
- Blood loss (uncommon)
What are the causes of decreased red cell production?
- Iron deficiency anaemia
2. Red cell aplasia
What are the different causes of red cell aplasia?
- Congenital red cell aplasia
- Transient erythroblastopenia of childhood (TEC)
- Parvovirus B19 infection
What are the causes of increased red cell destruction?
- Hereditary spherocytosis
- Thalassaemia
- Isoimmune
- Immune haemolytic anaemia
- G6PD
- Sickle cell disease
How is sickle cell disease inherited?
Autosomal dominant
What are the complications of sickle cell disease?
- Bacterial infection
- Acute chest syndrome
- Priapism
- Strokes
How is G6PD inherited?
X-linked, therefore usually affects males, but can affect females
What are the precipitants of haemolysis in G6PD?
- Infection
- Certain drugs
- Fava beans (broad beans)
- Naphthalene in mothballs
How is hereditary haemolysis treated?
Folic acid + splenectomy if symptomatic
How is hereditary haemolysis diagnosed?
Blood film
How is hereditary haemolysis inherited?
Autosomal dominant, but in 25% of cases there is no FHx
What are children with B-thalassaemia unable to produce?
HbA due to a mutation of the B-globulin gene
How is B-thalassaemia treated?
Regular blood transfusion + prevention of iron-overload, with iron-chelation therapy with desferrioxamine or iron chelation
What proportion of unilateral retinoblastomas are hereditary?
20%
On what chromosome is the gene that predisposes a child to retinoblastoma?
Chromosome 13
How do retinoblastoma present?
- White reflex
2. Squint
How is retinoblastoma managed?
Chemotherapy (for shrinkage) + laser treatment to the retina
Advanced cases = enucleation or radiotherapy
What is another name for nephroblastoma?
Wilm’s tumour
From where do Wilm’s tumours originate?
Embryonal renal tumour
What is the typical presentation of a Wilm’s tumour?
Large abdominal mass in an otherwise well child
How is a Wilm’s tumour investigated?
USS or CT/MRU KUB
How is a Wilm’s tumour treated?
Initial chemotherapy + delayed nephrectomy
Radiotherapy in advanced disease
From where do neuroblastomas arise?
From neural crest tissue in adrenal medulla and sympathetic nervous system - most commonly children present with an abdominal mass, but they can occur anywhere along the sympathetic chain from neck to pelvis
What is the most common leukaemia in children?
Acute Lymphoblastic Leukaemia - 80% of cases
How is ALL Dx?
FBC + bone marrow examination
What are the causes of bruising when the platelet count is REDUCED?
- Increased platelet destruction
2. Impaired platelet production
What are the causes of bruising when the platelet count is NORMAL?
- Platelet dysfunction
2. Vascular disorders
What are the causes of platelet dysfunctions that can cause bruising?
CONGENITAL - rare disorders, e.g. Glanzmann thromboasthenia
ACQUIRED - uraemia; cardiopulmonary bypass
What are the vascular disorders that can cause bruising?
CONGENITAL - Ehlers Danlos; Marfan’s; HHT
ACQUIRED - Meningococcal infection; HSP; SLE; scurvy
What are the causes of increased platelet destruction?
IMMUNE - ITP; SLE; alloimmune neonatal thrombocytopenia
NON-IMMUNE - haemolytic uraemic syndrome; TTP; CID; congenital heart disease; giant haemangiomas; hypersplenism
What are the causes of impaired platelet production?
CONGENITAL - Fanconi anaemia; Wiskott-Aldrich syndrome; Bernard-Soulier syndrome
ACQUIRED - Aplastic anaemia; marrow infiltration; drug-induced
What is the commonest cause of thrombocytopenia in children?
ITP
What are the atypical features one should be aware of when considering a Dx of ITP, as they may point to aplastic anaemia or acute leukaemia?
- Anaemia
- Neutropenia
- Hepatosplenomegaly
- Marked lymphadenopathy