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Paediatrics > Haematology > Flashcards

Haematology Flashcards

1
Q

Name 3 types of anaemia

A

Iron deficiency
Haemolysis - membrane/enzyme/immune
Haemoglobinopathies
Aplasia

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2
Q

Name a type of white blood cell haematology problem

A

Leukaemia

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3
Q

Name 2 causes of thrombocytopenia

A

Immune-ITP
Neonatal autoimmune
Marrow failure

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4
Q

Name 2 problems with clotting factors

A

Haemophilias

Hypercoagulable states

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5
Q

What is the normal range for Hb, MCV and WBC at birth?

A 
Hb = 149-237
MCV = 100-135
WBC = 10-26
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6
Q

What is the normal range for Hb, MCV and WBC at 2 weeks?

A 
Hb = 134-198
MCV = 88-120
WBC = 6-21
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7
Q

What is the normal range for Hb, MCV and WBC at 2 months?

A 
Hb = 94-130
MCV = 84-105
WBC = 6-18
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8
Q

What is the normal range for Hb, MCV and WBC between 2 and 6 years old?

A 
Hb = 115-135
MCV = 75-87
WBC = 5-17
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9
Q

What is the normal range for Hb, MCV and WBC between 7-12 years old?

A 
Hb = 115-155
MCV = 77-95
WBC = 4.5-14.5
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10
Q

What is the normal range for Hb, MCV and WBC between 12 and 18 years old in boys?

A 
Hb = 120-160
MCV = 78-95
WBC = 4.5-13
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11
Q

What is the normal range for Hb, MCV and WBC between 12-18 for girls?

A 
Hb = 130-160
MCV = 78-95
WBC = 4.5-13
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12
Q

Why is there a drop in normal Hb level at 2 months?

A

The HbF production has stopped but adults Hb production has not reached full level so there is a gap

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13
Q

What happens with haematopoiesis from birth to childhood?

A

Production of blood cells varies with age
By birth, virtually all bone marrow cavities are actively haematopoietic
In childhood, haematopoiesis moves to central bones - vertebrae, sternum, ribs, pelvis

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14
Q

What to pluripotent stem cells develop into in terms of haematology?

A

Mature erythrocytes
Monocytes
Megakaryocytes
Lymphocytes

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15
Q

What is stem cell development regulated by?

A

Cytokines

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16
Q

What is important in differentiating between anaemia types?

A

Mechanism
Red cell size/colour
Aetiology
Reticulocyte count

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17
Q

What mechanisms can lead to anaemia?

A

Decreased production
Increased consumption
Increased loss

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18
Q

What are the different sizes of red blood cells in anaemia?

A

Microcytic
Macrocytic
Normocytic

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19
Q

What are the different colours of red blood cells in anaemia?

A

Hypochromic

Normochromic

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20
Q

What are the 2 aetiologies associated with anaemia?

A

Congenital

Acquired

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21
Q

What are the 2 types of reticulocyte count and what do they mean?

A 
Low = lack of production
High = haemolysis/blood loss
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22
Q

What types of anaemia are related to low reticulocyte count?

A

Hypochromic, microcytic
Normochromic, microcytic
Macrocytic

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23
Q

What can cause hypochromic microcytic anaemia?

A 
Iron deficiency
Thalassaemia
Chronic inflammatory disease
Copper deficiency
Sideroblastic anaemia
Aluminium, lead intoxication
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24
Q

What can cause iron deficiency anaemia?

A

Chronic blood loss
Poor diet
Cow’s milk protein intolerance
Menstruation

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25
Q

What can cause normochromic normocytic anaemia?

A 
Chronic inflammatory disease
Recent blood loss
Malignancy/marrow infiltration
Chronic renal failure
Transient erythroblastopenia of childhood
Marrow aplasia/hypoplasia
HIV infection
Haemophagocytic syndrome
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26
Q

What can cause chronic inflammatory disease?

A

Infection
Collagen-vascular disease
IBD

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27
Q

What can cause macrocytic anaemia?

A 
B12/folate deficiency
Hypothyroidism
Oroticaciduria
Chronic liver disease
Lesch-Nyhan syndrome
Down syndrome
Marrow failure
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28
Q

What can cause B12 deficiency?

A 
Pernicious anaemia
Ileal resection
Strict vegetarian
Abnormal intestinal transort
Congenital intrinsic factor or transcobalamin deficiency
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29
Q

What can cause folate deficiency?

A 
Malnutrition
Malabsorption
Antimetabolite
Chronic haemolysis
Phenytoin
Tremethoprime
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30
Q

What are the 2 causes of raised reticulocyte count?

A

Loss

Haemolysis

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31
Q

What can cause RBC loss?

A

Bleeding
Burns
Splenomegaly - overactive

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32
Q

What are the two types of haemolysis?

A

Intrinsic - which part of the RBC is faulty?

Extrinsic - what’s causing destruction?

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33
Q

What are the causes of intrinsic haemolysis?

A

Membranopathies - hereditary spherocytosis, elliptocytosis, ovalocytosis
Enymopathies - G6PD deficiency, pyruvate kinase deficiency
Haemoglobinopathies - sickle cell disease, thalassaemias

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34
Q

What are the causes of extrinsic haemolysis?

A

Immune - autoimmune, alloimmune (transplant/haemolytic disease of the newborn), drug-induced
Others - DIC, HUS, TTP, burns, Wilson’s disease, vitamin E deficiency

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35
Q

What can cause severe anaemia at birth?

A

Haemolytic disease of the newborn

Bleeding from umbilical cord, internal haemorrhage

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36
Q

What is erythroblastosis fetalis?

A

Rh negative mother previously sensitised to Rh +ve cells
Transplacental passage of antibodies
Haemolysis of Rh +ve foetal cells

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37
Q

What are the S&S of erythroblastosis fetalis?

A

Severe anaemia

Compensatory hyperplasia and enlargement of blood forming organs - spleen and liver

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38
Q

What is the treatment of erythroblastosis fetalis?

A

Prevention of sensitisation with Rh immune globin

Intrauterine transfusion of affected foetus

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39
Q

What causes physiological anaemia of the newborn?

A

Fall in Hb from birth

  • Decreased RBC production
  • Plasma dilution associated with increasing blood volume
  • Shorter lifespan on neonatal RBCs (50-70 days)
  • More fragile RBCs
  • Switch from HbF to HbA - decreases around 3% per week, greater unloading of O2 to tissues for HbF as higher affinity for O2 than HbA
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40
Q

What causes anaemia of prematurity?

A

Low birth weight infants have poor erythropoietin response
Protein content of breast milk may not be sufficient for haematopoiesis in the premature infant
Hb level rapidly declines after birth to a low of 7-10g/dl at 6 weeks

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41
Q

What are the S&S of anaemia of prematurity?

A 
Apnoea
Poor weight gain
Pallor
Decreased activity
Tachycardia
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42
Q

Why is iron deficiency so common in children?

A

Poor intake and increased requirement

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43
Q

What can cause iron deficiency anaemia?

A

Breast feeding

Infection

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44
Q

Where do children get iron from?

A 
Iron from vegetables poorly absorbed
- Only 1% from rice and spinach
- 5% from wheat
- 10% from soya beans
- 10-20% from animal sources
- Up to 30% of iron in animal sources can be absorbed in latent iron deficiency
Children - 30% diet, 70% recycled red cells
Adults - 5% diet, 95% recycled red cells
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45
Q

What are the most common causes of iron deficiency anaemia in childhood?

A

Low birth weight
Dietary - excessive cow’s milk intake
Occult GI bleed eg hookworm
Cow’s milk intolerance

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46
Q

How does iron deficiency anaemia present?

A 
Pallor
Irritability
Anorexia when Hb < 50
Tachycardia
Cardiac dilatation
Murmur
Possibly splenomegaly
47
Q

What does the blood film in iron deficiency anaemia look like?

A

Microcytic
Hypochromic
Low-normal reticulocytes

48
Q

What do the iron bloods look like in IDA?

A

Low ferritin and serum iron
Increased TIBC
High ZPP

49
Q

How do you treat IDA?

A

Oral therapy mainstay
- 6mg/kg/day of elemental iron
- Reticulocytosis in 72hr, Hgb responds at 10g/L per week, iron stores replenished by 3 months
- Treatment 3-6 months to prevent relapse
- Constipation common S/E if not responding check that they are taking iron as sometimes won’t due to S/E
- Address cause
IV iron if enteral not possible
RBC only if unstable - v rare needed

50
Q

What does haemolysis lead to?

A

Increased RBC turnover
Shortened RBC lifespan
RBC’s fragile - especially abnormal ones

51
Q

What is the pathology of haemolysis?

A

Spleen filters out and breaks down senescent RBCs, and must work overtime, and can result in effective asplenia (sickle cell)
RBC degradation products must be handled

52
Q

What can lead to intra-corpuscular increased destruction of RBCs?

A

Haemoglobinopathies
Enzymopathies
Membranopathies

53
Q

What can lead to extra-corpuscular increased destruction of RBCs?

A

Autoimmune
Fragmentation
Hypersplenism
Plasma factors

54
Q

Name 2 types of haemoglobinopathies

A

Sickle cell disease
Thalassaemia
(Both recessive)

55
Q

What is normal haemoglobin like?

A

HbA - 2x alpha, 2x beta

HbF - 2x alpha, 2x gamma

56
Q

What does sickle cell haemoglobin look like?

A

HbSS

57
Q

What does sickle cell carrier haemoglobin look like?

A

HbSA

58
Q

What does severe sickled beta thalassaemia haemoglobin look like?

A

HbSbeta0

59
Q

How are haemoglobinopathies diagnosed?

A

High performance liquid chromatography or Hb electrophoresis

60
Q

How is sickle cell anaemia diagnosed?

A

FHx key
Antenatal and neonatal screening by Hb electrophoresis
Followed by specialist

61
Q

What interventions should you make sure are given to children with sickle cell disease?

A

Pneumococcal, influenza, meningococcal vaccines and prophylactic penicillin as functional asplenia therefore high risk for sepsis

62
Q

What is sickle cell disease?

A

Multi-system disease with severe and chronic complications

63
Q

What problems can occur with sickle cell disease?

A

Anaemia - cardiomegaly (high output), low pulse Ox, high WBC
Infarction - low O2 -> sickling due to Hb structure changes, pain crises, strokes
Infection/sepsis - asplenia from filtering abnormal RBCs, fever a serious sign
Splenic sequestration
Acute chest - infection/infarction
Aplastic crisis - parvovirus B19 infection
Iron overload - need for chelation
Stem cell transplantation - curative if good donor found
Reserved for severe cases

64
Q

What blood results might you get in sickle cell disease?

A

Hb 55-95g/L
Reticulocyte count raised 12%
Chronic anaemia, elevated WBC count, increases with vaso-occlusive event to 18-22 (in absence of fever)

65
Q

What should you do with a fever in a sickle cell patient?

A 
Serious, patient seek help for any fever
Seek source, blood cultures, CXR
IV fluids, antipyretics
Hospitalise for any pneumonia
Outpatient if not toxic, reliable family, follow up of cultures
66
Q

What should you do for pain in a sickle cell patient?

A

Frequent occurrence, treat mild with paracetamol and NSAIDs, patient and family know pain patterns
Trust patient and family and treat pain
Fluids, pain control - analgesia may need intranasal/IV morphine strong opiates
O2 if required
+/- IV fluids - dehydration can make pain worse

67
Q

What should you do for acute chest syndrome in a sickle cell patient?

A

Infection/infarction
25% of premature deaths
25% after surgery
Signs - pain crisis, respiratory distress, hypoxia, fever, neurological manifestations
Admit - avg around 10 day stay, 2-3 days in ICU
Aggressive physiotherapy/spirometry
Transfusion (top-up/exchange), IV fluids but avoid overload, O2, antibiotics, incentive spirometry

68
Q

What should you do for a stroke in a sickle cell patient?

A

Long-term transfusion therapy exchange

Will need chelation for iron overload if transfused > 1 year

69
Q

What should you do for aplastic crisis in a sickle cell patient?

A

Remember parvovirus B19 - switches off bone marrow RBC production so become severely anaemic quite quickly due to lower RBC lifespan
Can be post-op, need good hydration, O2
Low reticulocytes

70
Q

What should you do for splenic sequestration in a sickle cell patient?

A

Blood can pool in spleen causing hypovolaemia

Fluids - transfuse only to 80 or 90 g/L

71
Q

How can you treat sickle cell disease?

A

Hydroxycarbamide
- Increases HbF which carries O2 at lower O2 tension, good efficacy but teratogenic effects in pregnancy
Transfusion programmes - prevent strokes for those at highest risk
Stem cell transplants
- Patients with multiple strokes, frequent crises, if long term transfusion therapy needed possible GVHD
Gene therapies, other new treatments
Prevention of crises and infection

72
Q

What is the prognosis of sickle cell disease?

A

Prevention of crises and infection

73
Q

What is thalassaemia?

A

Reduced globin chain synthesis

74
Q

Why do babies often present later with beta thalassaemia?

A

HbF contains no beta chains only alpha and gamma chains

75
Q

How can carriers present and what can be a differential for this?

A

Mild microcytic hypochromic anaemia

Iron deficiency

76
Q

What is the presentation of alpha thalassaemia?

A

Loss of 1/2 asymptomatic
Loss of 3/4 - HbH
Loss of 4/4 death in utero - incompatible with life

77
Q

How does beta thalassaemia minor present?

A

Asymptomatic
Mild anaemia
Low MCV
Raised Hb A2

78
Q

How does beta thalassaemia major present?

A 
Progressive severe anaemia, low MCV, HbF and A2 increased
Jaundice
Splenomegaly
Failure to thrive
Skeletal deformity
Delayed puberty
Death early teens/adulthood
Regular blood transfusions mainstay of treatment
79
Q

How is beta thalassaemia treated?

A 
Genetic counselling, AN diagnosis
Regular transfusions
Complications of iron overload - liver, heart, pancreas, endocrinopathy - prevented by iron chelation
Bone marrow transplant
Genetic therapies
80
Q

What is transfusion dependent thalassaemia, how does it present?

A

Progressive severe anaemia requiring frequent transfusions
Bone marrow expansion to compensate, extramedullary haematopoiesis
Can also get hepato and splenomegaly

81
Q

What is iron overload?

A

Long term haemolysis and/or transfusions leading to iron overload affecting all organs
Ferritin to monitor and imaging
Chelation when necessary

82
Q

How does haemolytic anaemia present?

A 
Hydrops fetalis
Neonatal hyperbilirubinaemia
Neonatal ascites
Anaemia/failure to thrive
Splenomegaly
Cholecystitis/gall stones
Hyperbilirubinaemia
Leg ulcers
Aplastic crisis
Thromboembolism
83
Q

What is G6PD?

A

X-linked recessive enzymopathy

Glucose-6-phosphate dehydrogenase deficiency

84
Q

How does G6PD present?

A

Neonatal jaundice
Chronic non-spherocytic haemolytic anaemia
Intermittent episodes of intravascular haemolysis
Acute sever haemolysis

85
Q

What is acute severe haemolysis?

A

Intravascular haemolysis typically induced by drugs, fava (broad) beans, fever, acidosis

86
Q

How does acute severe haemolysis present?

A

Haemoglobinuria
Rigors
Fever
Back pain

87
Q

How is acute severe haemolysis treated?

A

Stop precipitant
Transfusion
Renal support

88
Q

How common is hereditary spherocytosis and how does it present?

A

Commonest hereditary anaemia in Europeans
1/5000
Typically autosomal dominant but no family history in 25% cases
Heterogeneous - deficiencies of spectrin, ankyrin
Clinical effects vary from mild to transfusion dependent - similar within families

89
Q

What is marrow failure?

A

Impaired production

90
Q

What can cause marrow failure?

A

Parvovirus
Transient erythroblastopenia of childhood
Diamond-blackfan anaemia (usually < 1)
Inherited/congenital - Fanconi’s anaemia, dyskeratosis
Acquired aplastic anaemia
Bone marrow infiltration - leukaemia/lymphoma, neuroblastoma, other solid tumours, Langerhan’s cell histiocytosis, osteopetrosis

91
Q

How does diamond-blackfan anaemia present?

A 
Craniofacial abnormalities
Thumb abnormalities 10-20%
Deafness
MSK
Renal
Cardiac
Growth retardation
92
Q

What are the symptoms of fanconi anaemia and what is it?

A

Autosomal/x linked recession - chromosomal fragility
Bone marrow failure, leukaemia, solid tumours
Absent/abnormal thumbs, cafe au lait patches, growth failure

93
Q

What are the requirements for haemostasis?

A

Platelets - numbers and function
Coagulation factors
Vascular integrity

94
Q

What are the two types of ITP?

A

Acute and chronic - acute often following viral illness and resolving in 1-3 months
Acute rarely needs treatment as recover spontaneously in weeks to months
Chronic may have associated bleeding eg GI, nose, gingivae

95
Q

In whom does ITP most commonly occur?

A

Young children

96
Q

Name 2 bleeding disorders

A

Haemophilia

VWD

97
Q

Name 2 hypercoagulable states

A 
Antithrombin
Protein C
Protein S
FVL
PT mutation
APS
98
Q

What is VWD and what is the pathology behind it?

A

Bleeding disorder caused by abnormalities of vWF - carrier protein for factor VII
Can range from almost undetectable to severe bleeding propensity
vWF binds on platelets to its specific receptor glycoprotein Ib and acts as an adhesive bridge between the platelets and damaged subendothelium at the site of vascular injury - causes platelets to stick
vWF also protects FVIII from degradation

99
Q

What are the different types of vWD and how do they present?

A

T1 (70-80%) - quantitative deficiency
- Autosomal dominant, variable penetrance
- Generally mild, can be asymptomatic and vary with time
T2A and 2B (15%) have qualitatively abnormal vWF
- Autosomal dominant
- Moderate severity
T3 - most severe, low vWF and FVIIIc in plasma, vWF absent on platelets
- Autosomal recessive, consanguinity an issue

100
Q

What is the history of VWD like?

A

Often mild bleeding - bruising, epistaxis, primary menorrhagia

101
Q

What investigations should you do for VWD?

A

Clotting screen - may be normal/APTT increased, vWF and FVIII - variably decreased

102
Q

How is VWD treated?

A

For bleeds/surgery, tranexamic acid, DDAVP usually increases vWF and factor VIII, factor VIII/vWF plasma concentrates for severe

103
Q

What is haemophilia?

A

Deficiency of factor VII/IX, increased APTT
X linked recessive - boys
Prolonged bleeding
Muscle bleeds and joint bleeds - arthritis and deformity

104
Q

How is haemophilia treated?

A

Regular infusions of factor VIII/IX - complications of treatment

105
Q

How do neonates with haemophilia present?

A

FHx
Cephalohaematoma/ICH
Iatrogenic bleeding
Umbilical cord bleeding

106
Q

How do children in early childhood with haemophilia present?

A

Classically < 2 once mobile
Easy bruising/soft tissue haematomas
Mouth bleeds - often trauma related
Muscle/joint bleeds

107
Q

How do moderate/mild cases of haemophilia present?

A

May present later following trauma/bleeding with surgery/dental extractions
Depends on level of factor

108
Q

What is the most common type of leukaemia in children?

A

ALL 85%

AML 13%

109
Q

What are the symptoms of ALL?

A 
Anaemia
Infection
Bleeding
Systemic symptoms
Organ infiltration
2/3 have symptoms for < 4 weeks
May have had more non-specific prodrome of several months
110
Q

In what age group is ALL most common?

A

4-7

111
Q

What investigations should you do for ALL?

A

Blood count - anaemia, WCC up or down, neutropaenia, thrombocytopaenia, blast cells
Bone marrow aspirate
LP

112
Q

What is the prognosis of ALL and what are the good prognostic factors?

A 
Prognosis > 90% cure
Good prognostic factors
- Age 2-10
- Female
- WCC < 50
- No CNS disease
113
Q

What is the treatment for ALL?

A 
Stratified into risk groups A B C
Induction
Consolidation and CNS treatment
Intensification
Maintenance girls 2 years, boys 3 years
114
Q

What are the late effects of treatment for leukaemia in children?

A 
Psychological
Family and social
Growth particularly CNS RT
Endocrine
Puberty
Fertility
Intellectual
Second malignancies

Paediatrics (21 decks)

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