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Paediatrics > Haematology > Flashcards

Haematology Flashcards

1
Q

Describe the changes in Hb levels from birth

A
  • The Hb concentration is high at birth (>140 g/L)

- Hb levels fall to the lowest level at around 2 months of age (about 100 g/L)

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2
Q

What is the definition of anaemia?

A

Anaemia is defined as an Hb level below the normal range. The normal range varies with age, so anaemia can be defined as:

  • Neonate: Hb < 140 g/L
  • 1 month to 1 year: Hb < 100 g/L
  • 1 year to 12 years: Hb < 110 g/L
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3
Q

How can the causes of anaemia be categorised?

A
  • Reduced red cell production
  • Increased red cell destruction (haemolysis)
  • Blood loss (uncommon in children)
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4
Q

Describe the different mechanisms of reduced red cell production

A
  • Ineffective erythropoiesis (e.g. iron deficiency, the most common cause of anaemia)
  • Red cell aplasia: complete absence of red cell production
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5
Q

What are the causes of iron deficiency anaemia?

A
  • Inadequate intake of iron (common)

- Malabsorption

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6
Q

Why might an infant/child have inadequate intake of iron?

A
  • Delay in introduction of mixed feeding beyond 6 months of age
  • Diet with insufficient iron-rich foods
  • Infants should not be fed unmodified cow’s milk as its iron content is low and poorly absorbed
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7
Q

Describe the clinical presentation of iron deficiency anaemia

A
  • Pallor
  • Lethargy
  • Some children exhibit ‘pica’ - inappropriate eating of non-food materials
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8
Q

Describe the investigation of iron deficiency anaemia

A

FBC:

  • Microcytic anaemia
  • Low serum ferritin
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9
Q

Describe the management of iron deficiency anaemia

A
  • Dietary advice

- Supplementation with oral iron

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10
Q

What is haemolytic anaemia?

A

Reduced red blood cell lifespan/premature destruction of RBCs

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11
Q

How can the causes of haemolytic anaemia be classified?

A
  • Red cell membrane disorders, e.g. hereditary spherocytosis
  • Red cell enzyme disorders, e.g. G6PD deficiency
  • Haemoglobinopathies, e.g. sickle cell disease, thalassaemia
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12
Q

Describe the epidemiology of sickle cell disease

A

More common in patients of black African/Caribbean descent

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13
Q

Describe the pathophysiology of sickle cell disease

A

Sickle cell disease is caused by defective Hb

  • In normal physiology, adult Hb is made up of two alpha-globin chains and two beta-globin chains
  • In sickle cell disease, there is a mutation in the beta-globin gene, which causes RBCs to become misshapen (sickled) when deoxygenated
  • This results in damage to red blood cell membranes and premature destruction (haemolytic anaemia)
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14
Q

Describe the inheritance of sickle cell disease

A

Autosomal recessive

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15
Q

What evolutionary advantage do sickle cell carriers have?

A

Decreased severity of Plasmodium falciparum malaria

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16
Q

Describe the clinical presentation of sickle cell disease

A
  • Anaemia (pallor, lethargy)
  • Increased susceptibility to infection (due to hyposplenism, secondary to chronic sickling and microinfarction of the spleen)
  • Painful, vaso-occlusive crises, e.g. dactylitis, avascular necrosis of bone, acute chest syndrome
17
Q

Describe the investigation of sickle cell disease

A
  • Newborn screening (heel-prick test)
  • FBC: raised reticulocyte count
  • Blood film: sickled erythrocytes
  • Hb electrophoresis
18
Q

Describe the conservative, pharmacological and interventional management of sickle cell disease

A

Conservative:
- Vaso-occlusive crises minimised by avoiding exposure to cold, dehydration, hypoxia, excessive stress/exercise

Pharmacological:

  • Immunisation and prophylactic antibiotics
  • Folic acid (increased demand)
  • Acute, painful crises managed with analgesia, fluids and oxygen
  • Disease modifying treatment: hydroxycarbamide

Interventional:
- Bone marrow transplant

19
Q

Describe the pathophysiology of beta thalassaemia

A

Beta thalassaemia is caused by defective Hb

  • In normal physiology, adult haemoglobin is made up of two alpha-globin chains and two beta-globin chains
  • In beta thalassaemia, there is a mutation in the beta-globin gene, which results in reduced or absent beta-globin chain synthesis
  • This results in damage to red blood cell membranes and premature destruction (haemolytic anaemia)
20
Q

Describe the inheritance of beta thalassaemia

A

Autosomal recessive

21
Q

Describe the investigation of beta thalassaemia

A
  • FBC: raised reticulocyte count
  • Blood film: microcytic, hypochromic RBCs
  • Hb electrophoresis
22
Q

Describe the management of beta thalassaemia minor, intermedia and major

A

Beta thalassaemia minor does not usually require treatment

Beta thalassaemia major (and sometimes intermedia):
- Regular blood transfusions

23
Q

What needs to be considered in a patient receiving regular blood transfusions?

A
  • Risk of iron overload and deposit in major/endocrine organs, e.g. liver, heart, pancreas
  • Therefore, iron chelation agents are given, e.g. deferoxamine, penicillamine
24
Q

What are the different types of haemophilia?

A
  • Haemophilia A (factor XIII deficiency) is most common

- Haemophilia B (factor IX deficiency)

25
Q

Describe the inheritance of haemophilia

A

X-linked recessive

26
Q

Describe the management of haemophilia

A
  • Haemophilia A: recombinant factor XIII concentrate

- Haemophilia B: recombinant factor IX concentrate

27
Q

Describe the pathophysiology of von Willebrand’s disease

A

Quantitative/qualitative deficiency in vWF, resulting in defective platelet plug formation

28
Q

Describe the inheritance of von Willebrand’s disease

A

Autosomal dominant

29
Q

What is the definition of thrombocytopenia?

A

Platelet count <150 x 10^9/L

30
Q

Describe the classification of thrombocytopenia

A
  • Severe: <20 x 10^9/L
  • Moderate: 20-50 x 10^9/L
  • Mild: 50-150 x 10^9/L
31
Q

Describe the clinical presentation of thrombocytopenia

A
  • Bruising
  • Petechiae
  • Purpura
  • Excessive, prolonged bleeding (particularly mucosal bleeding, e.g. epistaxis, menorrhagia)
32
Q

What is the most common cause of thrombocytopenia?

Describe its pathophysiology

A
  • Immune thrombocytopenic purpura (ITP)
  • Caused by destruction of platelets by IgG antibodies
  • Usually occurs following viral infection
33
Q

Describe the prognosis and management of ITP

A
  • Self-limiting

- Doesn’t usually require treatment

34
Q

Which type of leukaemia is most common in childhood?

A

Acute lymphoblastic leukaemia (ALL)

35
Q

Describe the clinical presentation of ALL

A
  • Usually present 2-5 years
  • In most children, ALL presents insidiously over several weeks, e.g. malaise, anorexia
  • Signs of bone marrow infiltration:
    Anaemia - pallor, lethargy
    Neutropenia - increased susceptibility to infection
    Thrombocytopenia - bruising, bleeding (e.g. epistaxis), petechiae
    Bone pain
  • Other signs:
    Hepatosplenomegaly
    Lymphadenopathy
36
Q

Describe the investigation of ALL

A
  • FBC - anaemia, neutropenia, thrombocytopenia
  • Blood film - blast cells
  • Bone marrow biopsy is diagnostic
37
Q

Describe the management of ALL

A
  • Anaemia - blood transfusion
  • Thrombocytopenia - platelet transfusion
  • Neutropenia - treat any infection
  • Chemotherapy and steroids

Paediatrics (17 decks)

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