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Paediatrics > Haematology > Flashcards

Haematology Flashcards

1
Q

What is the most common malignancy of childhood?

A

ALL

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2
Q

What is the peak incidence of ALL?

A

2-5 years

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3
Q

Which conditions increase the risk of developing ALL?

A
  • Down’s syndrome
  • Kleinfelter syndrome
  • Noonan syndrome
  • Fanconi’s anaemia
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4
Q

What are the clinical features of ALL?

A
  • Anaemia: fatigue, pallor
  • Neutropoenia: frequent and severe infections
  • Thrombocytopaenia: petechiae and abnormal bruising
  • Other features:
    i. unexplained fever
    ii. failure to thrive/weight loss
    iii. night sweats
    iv. abdominal pain
    v. hepatosplenomegaly
    vi. generalised lymphadenopathy
    vii. unexplained or persistent bone/joint pain
    viii. testicular enlargement (rare - but ALL commonly spreads to CNS and testes)
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5
Q

What investigations should be performed if ALL is suspected?

A
  1. FBC: anaemia, thrombocytopaenia, raised WBCs
  2. Peripheral blood smear: presence of blasts
  3. Bone marrow biopsy: >20% lymphoblasts
  4. Lymph node biopsy
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6
Q

What investigations are done to ‘stage’ ALL?

A
  • CXR
  • CT
  • LP (CNS metastases)
  • Genetic analysis and immunophenotyping
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7
Q

Describe the management of ALL.

A
  • Chemotherapy (CVAD - cyclophosphamide; vincristine; adriamycin/daunorubicin; dexamethasone)
  • ± radiotherapy
  • ± bone marrow transplant
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8
Q

What is the survival rate of children with ALL?

A

5 year survival rate ~80%

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9
Q

What factors indicate a poor prognosis for ALL?

A
  • Age <2 years or >10 years
  • WBC >20 x 109/L at diagnosis
  • T or B cell surface markers
  • Non-Caucasian
  • Male sex
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10
Q

What are the causes of microcytic anaemia in children?

A

TAILS

  • Thalassaemia
  • Anaemia of chronic disease
  • Iron deficiency anaemia
  • Lead poisoning
  • Sideroblastic anaemia
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11
Q

What are the causes of normocytic anaemia in children?

A

AAAHH

  • Acute blood loss
  • Anaemia of chronic disease
  • Aplastic anaemia
  • Haemolytic anaemia
  • Hypothyroidism
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12
Q

What are the causes of megaloblastic macrocytic anaemia in children?

A
  • B12 deficiency

- Folate deficiency

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13
Q

What are the causes of normoblastic macrocytic anaemia in children?

A
  • Reticulocytosis (usually due to haemolytic anaemia or blood loss)
  • Hypothyroidism
  • Liver disease
  • Drug-induced (azathioprine)
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14
Q

What are the causes of red cell aplasia in children?

A
  • Parvovirus B19
  • Diamond-Blackfan anaemia (congenital red cell aplasia)
  • Rarer causes: Fanconi anaemia, aplastic anaemia, leukaemia
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15
Q

How can reticulocyte be used to determine the cause of anaemia?

A
  • Reticulocyte count low: anaemia likely to be due to issue with red cell production, i.e. red cell aplasia
  • Reticulocyte count high: anaemia likely to be due to increased RBC destruction or blood loss
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16
Q

What clinical features are specific to iron deficiency anaemia?

A
  • Pica
  • Hair loss
  • Koilonychia
  • Angular cheilitis
  • Atrophic glossitis
  • Brittle hair and nails
17
Q

What is the inheritance pattern of G6PD deficiency?

A

X-linked recessive

18
Q

What are the clinical features of G6PD deficiency?

A
  • Neonatal jaundice and jaundice in response to triggers
  • Anaemia
  • Splenomegaly
  • increased risk of developing gallstones
19
Q

What is a typical finding on blood film in G6PD deficiency?

A

Heinz bodies –> blobs of denatured haemoglobin (inclusions) within RBCs

20
Q

What medications such be avoided in G6PD deficiency?

A
  • Primaquine
  • Ciprofloxacin
  • Nitrofurantoin
  • Trimethoprim
  • Sulfonylureas
  • Sulfasalazine and other sulphonamides
21
Q

What is the inheritance pattern of hereditary spherocytosis?

A

Autosomal dominant

22
Q

What are the clinical features of hereditary spherocytosis?

A
  • Jaundice
  • Anaemia
  • Splenomegaly
  • increased risk of developing gallstones
23
Q

What is a common trigger for aplastic crisis in hereditary spherocytosis and what are the clinical features?

A

Trigger:
- Parvovirus

Features:

  • Anaemia
  • Haemolysis
  • Jaundice
24
Q

What is the management for hereditary spherocytosis?

A
  • Folate supplementation
  • Splenectomy
  • Cholecystectomy
  • Blood transfusion (aplastic crisis)
25
Q

State the typical results seen in iron deficiency anaemia of the following:

  1. Serum iron
  2. Ferritin
  3. TIBC
  4. Transferrin saturation
A
  1. Low (but will vary throughout the day - highest level in the morning and after meals)
  2. Low (but can be normal)
  3. Increased
  4. Low
26
Q

What is the inheritance pattern of sickle cell anaemia (SCA)?

A

Autosomal recessive

27
Q

In which chromosome is the defect in sickle cell anaemia?

A

11

28
Q

Describe the presentation of a vaso-occlusive crisis in SCA.

A

Cause:
- sickled RBCs clogging capillaries and causing distal ischaemia

Features:

  • pain
  • fever
  • signs of infection
  • dehydration
  • raised haematocrit
  • priapism
29
Q

Describe the presentation of a splenic sequestration crisis in SCA and its management.

A

Cause:
- RBCs blocking blood flow within the spleen

Features:

  • enlarged and painful spleen
  • severe anaemia
  • circulatory collapse

Management:

  • supportive: blood transfusions and fluid resuscitation
  • splenectomy (severe and measure to prevent recurrent crises)
30
Q

Describe the presentation of a acute chest syndrome in SCA and its management.

A

Causes:

  • infective: pneumonia, bronchiolitis
  • non-infective: vaso-occlusion or fat emboli

Features:

  • fever
  • resp symptoms
  • new infiltrates on CXR

Management:

  • treat infection (abx/antivirals)
  • blood transfusion
  • incentive spirometry
  • NIV or intubation if required
31
Q

What is the inheritance pattern of alpha and beta thalassaemia?

A

Autosomal recessive

32
Q

In which chromosome is the defect in alpha thalassaemia?

A

16

33
Q

In which chromosome is the defect in beta thalassaemia?

A

11

34
Q

What type of hypersensitivity reaction is idiopathic thrombocytopenic purpura (ITP)?

A

Type II –> production of antibodies that target and destroy platelets

35
Q

What are the clinical features of ITP?

A
  • Bleeding from the gums, epistaxis, or menorrhagia
  • Bruising
  • Petechial or purpuric rash
36
Q

What are the typical findings in FBC, clotting screen, prothrombin time in ITP?

A

Isolated thrombocytopenia (low platelets) - all other results are normal

37
Q

What is the management of ITP?

A

No treatment –> 70% will resolve spontaneously in <3 months

Treatment may be required if patient is actively bleeding, or if thrombocytopenia is severe:

  • Prednisolone
  • IVIG
  • Blood transfusions
  • Platelet transfusions

Paediatrics (14 decks)

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