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Paediatrics > Haematology > Flashcards

Haematology Flashcards

1
Q

Changes to haemoglobin synthesis in newborn

A

Decreasing production of Fetal Hb (2 alpha, 2 gamma)

Increasing production of adult Hb (HbA and HbA2)

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2
Q

Changes to haemoglobin concentration at birth

A

High at birth, falls to lowest level at 2 months

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3
Q

Anaemic values according to age group

A

Neonate: <140g/L
1month - 12 months: <100g/L
1 year - 12 years: <110g/L

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4
Q

Causes of anaemia in children

A
  1. Impaired red cell production
    - red cell aphasia (absence of production)
    - ineffective erythropoiesis: iron deficiency
  2. Increased red cell destruction
    - hereditary spherocytosis (membrane)
    - Thalassaemias (haemoglobinopathies)
    - G6PDH deficiency (enzyme)
    - autoimmune haemolytic anaemia
  3. Blood loss
    - coagulopathies
    - GI (meckel diverticulum)
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5
Q

Diagnostic approach to anaemia in children

A

A. Reticulocyte low:

  • red cell aplasia
  • causes: Parovirus B19, congenital
  • tests: Parovirus serology, bone marrow aspirate

B. Reticulocyte normal/high:

  • bilirubin raised
  • causes: hereditary spherocytosis, sickle cell, thalassaemia
  • tests: blood film, Hb electrophoresis
  • bilirubin normal
  • causes: blood loss, iron deficiency anaemia
  • tests: blood film, serum ferritin
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6
Q

Causes of iron deficiency anaemia in children

A

Inadequate intake:
Delay in introduction to mixed feeding beyond 6 months
Diet excess in cows milk

Blood loss
Malabsorption

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7
Q

Why is iron requirement high in children

A

Increase in blood volume accompanying growth

Need to build up iron stores

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8
Q

Content and absorption of iron from milk and solid food

A

Breast milk - low content but 50% absorbed
Infant formula - high iron content
Cows milk - low iron content and 10% absorbed
Mixed diet - high content

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9
Q

Sources of iron in diet

A

Red meat, oily fish
Pulses, fortified breakfast cereals, dark green veg, dried fruit, nuts
Vitamin c increases iron absorption
Tea and Fibre decreases iron absorption

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10
Q

Clinical features of iron deficiency anaemia

A

Asymptomatic until <60g/L
Feed slowly
Tire easily
‘Pica’ - inappropriate eating of non-food materials
Behavioural and intellectual deficiencies

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11
Q

Investigations for iron deficiency anaemia

A

FBC - Low Hb, Low MCV and MCH, microcytic hypochromic red cells
Serum ferritin - Low

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12
Q

Differentials for iron deficiency anaemia

A

Beta thalassaemia trait

Anaemia of chronic disease

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13
Q

Management of iron deficiency anaemia

A

Dietary advice

Iron Supplement - Styron, niferex

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14
Q

Duration of iron supplement treatment

A

Until Hb normal then minimum of further 3 months

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15
Q

Treatment if iron deficiency with normal Hb

A

Dietary advice and offer option of iron supplements

  • risk of iron toxicity w treatment
  • risk of behaviour and intellectual function with iron deficiency
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16
Q

Define thrombocytopenia

A

Platelet count <150x10^9/L

17
Q

Severity of thrombocytopenia and their risk of bleeding

A

Severe: <20, risk of spontaneous bleeding
Moderate: 20-50, risk of severe bleeding during operation/trauma
Mild: 50-150, low risk of bleeding unless major operation/trauma

18
Q

Clinical features of thrombocytopenia

A 
Petechiae 
Purpura
Bruising
Mucosal bleeding - epistaxis, gum 
Severe bleeding - GI, intracranial
19
Q

Differentials for purpura/bruising

A

A. Thrombocytopenia

  1. increased destruction
    - Immune: ITP, SLE
    - Non-Immune: DIC, CHD, Hypersplenism, TTP
  2. decreased production
    - aplastic anaemia
    - leukaemia, marrow infiltration

B. Platelet dysfunction
- uraemia

C. Vascular disorders

  • congenital: Ehlers Danlos, Marfans
  • meningococcal infections
  • vasculitis
20
Q

Define immune thrombocytopenia

A

Autoimmune destruction of platelets by antiplatelet IgG autoantibodies

21
Q

Prevalence of ITP

A

Most common cause of thrombocytopenia in children

22
Q

Clinical features of ITP

A

2-10 years
Onset 1week after viral infection
Features of thrombocytopenia

23
Q

Investigations for ITP

A

Diagnosis of exclusion - isolated thrombocytopenia in absence of other causes
FBC
Blood film
Bone marrow - to exclude lEukaemia, aplastic anaemia; If starting steroids to rule out ALL

24
Q

Management of acute ITP

A

Self-limiting: resolves in 6 weeks

Supportive: 24hr access to hospital, avoid contact sports

Medical: if major / persistent bleeding
Oral prednisolone
IV anti-D
IV Immunoglobulin

Platelet transfusion - ONLY if life threatening haemorrhage

25
Q

Define chronic ITP

A

Thrombocytopenia for >6 months from diagnosis

Occurs in 20%

26
Q

Management of chronic ITP

A

Supportive

Medical: if major / persistent bleeding impacting QoL
Rituximab, monoclonal antibodies against B cells

Splenectomy - with life long antibiotics

Regular screening for SLE - May develop SLE

27
Q

Causes of leukaemia in childhood

A

Acute lymphoblastic leukaemia (80%)

Acute myeloid leukaemia

28
Q

What is ALL

A

Malignancy of lymphoid progenitor cell that leads to uncontrolled proliferation

29
Q

Clinical features of ALL

A

2-5 years
Insidious onset
General: malaise, anorexia
Lymphadenopathy, Hepatosplenomegaly (reticuloendothelial infiltration)
Lethargy, pallor, infection, bruising, petechiae, bone pain (Bone marrow infiltration)
headaches, vomiting (CNS infiltration)

30
Q

Investigations for ALL

A

FBC - anaemia, neutropenia, thrombocytopenia
Peripheral blood smear - leukaemic lymphoblasts
Bone marrow aspiration
Cytogenetic and immunological characteristics - prognosis

Clotting screen
LP - CNS infiltration
CXR - mediastinal lymphadenopathy

31
Q

Prognostic factors for ALL

A

Age <1, >10
WCC >50x10^9/L (tumour load)
Cytogenetic and immunological characteristics
Persistence of leukaemic lymphoblasts In bone marrow after chemotherapy
Detectable leukaemia after induction treatment

32
Q

Types of chemotherapy treatment for ALL

A 
Induction
Intensification 
Intrathecal
Maintenance 
Relapse
33
Q

What supportive treatments do you need to give prior to induction chemotherapy

A

Blood transfusion - if anaemic
Platelet transfusion - if thrombocytopenia
Treat infection
Allopurinol + hydration: protect renal function from rapid cell lysis

34
Q

What is the purpose of Intrathecal chemotherapy

A

To Prevent relapse of leukaemic cells in CNS

Bc Cytotoxic Drugs poorly infiltrate CNS and leukaemic cells may survive in CNS with systemic treatment

Paediatrics (15 decks)

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