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Year 4 Paediatrics > Paediatric Haematology > Flashcards

Paediatric Haematology Flashcards

1
Q

Feotal haemoglobin structure

A

2 alpha and 2 gamma units - greater affinity to oxygen

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2
Q

Hydroxycarbamide

A

Used to increase the production of HbF in sickle cell patients

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3
Q

Why do sickle cell patients present after 1 years old

A

Sickle cell involves a defect in the beta haemoglobin - before 1 years old, infants still have some HbF which has a greater affinity for oxygen

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4
Q

Causes of anaemia in infancy

A
  • Physiological
  • anaemia of prematurity
  • blood loss
  • haemolysis
  • twin to twin transfusion
  • haemolysis - spherocytosis, G6PD deficiency, haemolytic disease of the newborn
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5
Q

Physiological anaemia

A

Normal dip in haemoglobin between 6 - 9 weeks due to high oxygen delivery to the tissues causing negative feedback

Less erythropoietinproduction by the kidneys

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6
Q

Causes of anaemia of prematurity

A

Become anaemic due to:

  • neonatal sepsis
  • less time in utero recieving iron from mother
  • RBC production cannot keep up with rapid growth in first few weeks
  • Reduced erythropoietin levels
  • Frequent blood tests
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7
Q

Pathophysiology of haemolytic anaemia of the newborn

A

Caused by incompatibility between the rhesus antibodies of the mother and rhesus antigens of the baby

Occurs in a rhesus negative mother and rhesus positive second child

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8
Q

Test for haemolytic disease of the newborn

A

Direct Coombs test

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9
Q

Management of haemolytic disease of the newborn

A

Baby is given phototherapy if jaundiced

May need blood transfusion

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10
Q

Prevention of haemolytic disease of the newborn

A

Mothers are given RhoGAM during pregnancy which is a rhesus immunoglobulin

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11
Q

Causes of anaemia in older children

A

Iron deficiency - dietary insufficiency
Blood loss - menstruation in girls

Sickle cell anaemia
Thalassaemia 
Leukaemia
Hereditary spherocytosis or eliptocytosis 
Sideroblastic anaemia
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12
Q

Presentation of anaemia

A 
Fatigue and lethargy 
SOB on exertion
Headaches 
Dizziness 
Palpitations
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13
Q

Causes of microcytic anaemia

A

TAILS:

Thalassaemia 
Anaemia of chronic disease 
Iron deficiency 
Lead poisoning 
Sideroblastic anaemia
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14
Q

Causes of normocytic anaemia

A

3 As + 2 Hs

Anaemia of chronic disease
Acute blood loss
Aplastic anaemia

Haemolytic anaemia
Hypothyroidism

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15
Q

Causes of macrocytic anaemia

A

FAT - L

Folate and B12 deficiency
Alcohol
Thyroid disease - hypothyroidism
Liver disease

Others:

  • Azathioprine
  • Reticulocytosis
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16
Q

Symptoms specific to iron deficiency

A

Pica - weird dietary cravings

Hair loss

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17
Q

Signs of anaemia

A

Pallor
Tachycardia
Pale conjunctiva
Raised RR

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18
Q

Signs of iron deficiency

A

Koilonychia - spooned nails
Angular chelitis
Atrophic glossitis

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19
Q

Investigations for anaemia

A 
FBC 
Blood film if required 
Reticulocyte count 
Iron - ferritin 
B12 and folate 
Bilirubin- if jaundice or haemolysis suspected

Direct Coombs test - autoimmune haemolytic anaemia
Haemoglobin electrophoresis- haemoglobinopathies

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20
Q

Causes of iron deficiency

A

Inadequate dietary intake
Menstruation
Malabsorption - Crohn’s and Coeliacs, PPI use

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21
Q

Blood tests for iron

A 
Serum ferritin (Fe2+) 
Serum Iron - not useful on its own as varies throughout the day 
Total iron binding capacity - space on transferrin molecules for iron to bind 
Transferrin saturation ( serum iron/ TIBC)
22
Q

What increases free iron

A

Supplementation with iron
Acute liver damage - as iron is stored in the liver
Inflammation

23
Q

Side effects of iron supplements

A

Constipation

Black stools

24
Q

Leukaemia

A

Unregulated production of either myeloid or lymphoid blood cells due to cancer of the bone marrow

25
Types of leukaemia
Acute lymphoblastic leukaemia - most common in children Acute myeloid leukaemia Chronic myeloid leukaemia - rare
26
In which age groups are acute lymphoblastic leukaemia and acute myeloid leukaemia seen in?
Acute lymphoblastic leukaemia - 2 - 3 years old | Acute myeloid leukaemia - under 2 years old
27
Risk factors for leukaemia
``` Radiation exposure during pregnancy Down’s syndrome Kleinfelter syndrome Noonan syndrome Fanconi’s anaemia ```
28
Presentation of leukaemia
``` FLAWS - fever, lethargy, appetite loss, weight loss, sweating at night Failure to thrive Pallor - anaemia Petechiae + abnormal bruising and bleeding - thrombocytopenia Abdominal pain Generalised lymphadenopathy Joint or bone pain Hepatosplenomegaly ```
29
Diagnosing leukaemia
Refer any child with unexplained petechiae or hepatomegaly for immediate specialist assessment Urgent (within 48 hours) FBC - anaemia, thrombocytopenia, leukopenia Blood film - blast cells Bone marrow biopsy Lymph node biopsy Staging CT scan, chest Xray, LP, genetic analysis using imunnophenotyping
30
Management of leukaemia
MDT - paediatric oncologist - chemotherapy Other therapies: - radiotherapy - bone marrow transplant - surgery
31
Complications of chemotherapy
``` Failure to treat leukaemia Stunted growth and development Immunodeficiency- recurrent infections Neurotoxicity Infertility Secondary malignancy Cardiotoxicity ```
32
Prognosis for acute lymphoblastic leukaemia
80% cure rate
33
Pathophysiology of idiopathic thrombocytopenic purpura
Type II sensitivity reaction causing autoimmune destruction of plateletes
34
Presentation of idiopathic thrombocytopenic purpura
Presents in children under 10 years old often after a viral illness Symptoms present 1 - 2 days after virus: - bleeding - epistaxis, gums or menorrhagia - bruising - petechial or purpuric rash
35
Management of idiopathic thrombocytopenic purpura
If actively bleeding or severe thrombocytopenia (<10mmol/l) - prednisolone - IV immunoglobulins - blood transfusion if required - platelet transfusion if required Education: - avoid contact sport - avoid IM injections and lumbar punctures - avoid NSAIDs, aspirin and blood thinning medication - advice on managing nosebleeds - seek help after an injury that may cause internal bleeding
36
Investigations for idiopathic thrombocytopenic purpura
Bloods - FBC - thrombocytopenia | Blood culture to rule out sepsis
37
Complications of idiopathic thrombocytopenic purpura
Chronic idiopathic thrombocytopenic purpura (ITP) Anaemia Intracranial and subarachnoid haemorrhage GI bleeding
38
Sickle cell anaemia pathophysiology
Abnormal gene for beta haemoglobin on chromosome 11 (glut - valine substitution) causing sickling and haemoglobin S which is prone to haemolysis
39
Sickle cell trait
Carrier but not symptomatic
40
Sickle cell inheritance pattern
Autosomal recessive
41
How to diagnose sickle cell anaemia
Heel prick test at 5 days old | Testing during pregnancy for high risk mothers
42
Complications of sickle cell anaemia
Increased risk of infection Stroke Avascular necrosis especially in the hip Pulmonary hypertension Painful and persistent penile erection (priapism) CKD Sickle cell crisis
43
General management for sickle cell
Stay hydrated and avoid triggers for crises Ensure vaccinations are up to date Antibiotic prophylaxis Hydroxycarbamide - stimulates production of HbF Blood tranfusion - severe anaemia Bone marrow transplant
44
Types of crisis
Vaso - occlusive crisis (painful crisis) Splenic sequestration crisis Aplastic crisis
45
Vaso - occlusive crisis
HbS (sickled Hb) blocks capillaries and causes distal ischaemia - associated with dehydration and raised haematocrit Symptoms: - pain - fever - priapism
46
Management of priapism
Urological emergency | Treated with apiration of the blood from the penis
47
Splenic sequestration crisis
Caused by HbS blocking blood flow within the spleen causing acute and painful splenomegaly Pooling of blood within the spleen can cause severe anaemia and hypovolaemic shock Emergency - blood tranfusion and fluod resuscitation to treat anaemia and shock Recurrent crises - splenectony
48
Aplastic crisis
Temporary loss of the creation of new red blood cells - often due to parvovirus B19 Causes significant anaemia - often requiring blood transfusions. Can resolve spontaneously within a week
49
Acute chest syndrome
Fever/resp symptoms + new inflitrates seen on chest Xray Can be due to infection e.g. pneumonia or bronchiolitis or pulmonary vaso - occlusion
50
Treatment of acute chest syndrome
Emergency Antibiotics or antivirals Blood transfusion - anaemia Incentive spirometry - encourages deep breathing Artificial ventilation - NIV/ intubation

Year 4 Paediatrics (19 decks)

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