Paediatric haematology Flashcards
Important haematological conditions in children
Iron deficiency anaemia. Acute lymphoblastic leukaemia (ALL)
Sickle cell disease. Thalassaemia
Immune/idiopathic thrombocytopenic purpura (ITP). Haemophilia A
Definition of anaemia
A reduction of Hb below the normal range
Adult male - if <160g/L
Causes of anaemia
Failure of production (reticulocytes will be low)
Excessive loss from bleeding or haemolysis (reticulocytes high or normal)
Iron deficiency
Adults need 1mg/day iron — absorbed through duodenum
Depletion of iron stores (low ferritin) – anaemia (hypochromic and microcytic) leads to growth and developmental delay with stomatitis and kolionychia
Causes of iron deficiency in children
Dietary deficiency –> children are fussy, vegan/vegetarian as children are worse at absorbing non-haem iron, delayed weaning/large milk intake, poverty, coeliac disease, parasite infections (hookworm)
Treatment of iron deficiency anaemia in children
Dietary advice –> less milk and more meat, oral or IV iron supplements, if severe can need transfusions
Treat parasite infection and any other causes
Acute lymphoblastic leukaemia (ALL)
Commonest Ca in childhood – 500 new cases/yr
Diagnosis - blood count (anaemia, thrombocytopenia, neutropenia) – blood film shows blasts – lymphtyping shows lymphoblasts – marrow aspiration will show >30% blasts
Causes of Acute lymphoblastic leukaemia (ALL)
Usually idiopathic but increased risk if exposed to radiation, chemicals, EM fields
Increased risk if downs or Fanconi anaemia
Treatment of Acute lymphoblastic leukaemia (ALL)
Myeloablative chemotherapy – Intensive induction of remission –> followed by intensification blocks to kill any remaining cells –> followed by 2-3yrs of low dose OP maintenance treatment
This can be done intrathecally
Outcomes of ALL treatment
70-80% of children ‘cured’ - best prognosis if: female, between 1 and 16yrs, if WCC was low at presentation, rapid response, hyperdiploidy
Side effects of ALL treatment
Acute –> Naesea and vomiting, pancytopenia (bleeding and infection risk), hair loss
Long term –> Growth/cogntive problems, behavioural/psychological problems, cardiomyopathy, subfertility
ALL relapse
If relapse occurs further intensive therapy is started
consider bone marrow donor if remission occurs
Ideally – HLA identical or Haploidentical (sibling or parent)
Epidemiology of Sickle cell disease in children
10,000 children with SCD in london - 12,000 in UK
Most common and fastest growing serious genetic disease in Britain
Screened as part of heel prick and can now be screened antenatally
Symptoms of SCD in children
Pain from crises - acute and chronic
Stroke risk and protect cognitive function
Pulmonary HTN and acute chest syndrome
Priapism
Hydroxycarbamide
Reduces frequency of Sickle cell crises by increasing the production of fetal haemoglobin which prevents sickling
Thalassaemia
Quantitative defect in globin chain production – carriers are asymptomatic
Thalassaemia intermedia - some symptoms
Thalassaemia major - if Beta will die without regular transfusions, if alpha die in utero
Immune/idiopathic thrombocytopenic purpura (ITP)
Acquired, immune mediated destruction of platelets
Symptoms are purpura and bleeding
Diagnosed by isolated low platelet count
Megkaryocytes in marrow can be normal or increased
Treatment of ITP
80% of children will recover in 6months and life-threatening bleeds are rare
Treatment is supportive with immunoglobulins or corticosteroids - if severe may need splenectomy, other immunosuppresants or biologics
Wilms Tumour (Nephroblastoma)
Presents <5 (median 3) with abdominal mass (also haematuria, flank pain, anorexia, fever). Unilateral in 95% of cases. 20% will spread to lung. Treat with nephrectomy, chemotherapy and radiotherapy. 80% cure rate
Associations of Wilm’s tumour
Beckwith-wiedemann syndrome WAGR syndrome (Aniridia, GU malformations,mental retardation) Hemihypertrophy. 1/3 of cases are associated with a mutation of the WT1 gene on chr 11
Hand-foot syndrome
Vaso-occulusive crises in pre-school children with Sickle cell. This can be dactylyitis (fingers) or in the feet.
Asplenism
Can occur due to surgical removal, sickle cell or rarely coeliac. Can lead to Howell-jolly bodies (basophilic nuclear fragments), pappenheimer bodies and target cells (RBCs with peripherial depigmentation).
Acute Myeloid Leukaemia (AML)
Commonest acute leukaemia in adults - risk is increased in downs children (and for ALL).
Mild splenomegaly & fever/fatigue/weight loss/anorexia. Auer Rods are cellular inclusions in bone marrow blast cells which are pathognomic of AML.
Polycythaemia
Central Venous Haematocrit of more than 0.65. This hyperviscosity can present with lethargy, hypotonia, hyperbilirubinaemia, hypoglycaemia, seizures, stroke, renal vein thrombosis and NEC
