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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
State the typical results seen in iron deficiency anaemia of the following: 1. Serum iron 2. Ferritin 3. TIBC 4. Transferrin saturation
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
What is the inheritance pattern of sickle cell anaemia (SCA)?
Autosomal recessive
27
In which chromosome is the defect in sickle cell anaemia?
11
28
Describe the presentation of a vaso-occlusive crisis in SCA.
Cause: - sickled RBCs clogging capillaries and causing distal ischaemia Features: - pain - fever - signs of infection - dehydration - raised haematocrit - priapism
29
Describe the presentation of a splenic sequestration crisis in SCA and its management.
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
Describe the presentation of a acute chest syndrome in SCA and its management.
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
What is the inheritance pattern of alpha and beta thalassaemia?
Autosomal recessive
32
In which chromosome is the defect in alpha thalassaemia?
16
33
In which chromosome is the defect in beta thalassaemia?
11
34
What type of hypersensitivity reaction is idiopathic thrombocytopenic purpura (ITP)?
Type II --> production of antibodies that target and destroy platelets
35
What are the clinical features of ITP?
- Bleeding from the gums, epistaxis, or menorrhagia - Bruising - Petechial or purpuric rash
36
What are the typical findings in FBC, clotting screen, prothrombin time in ITP?
Isolated thrombocytopenia (low platelets) - all other results are normal
37
What is the management of ITP?
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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