Haematology - anaemia Flashcards

(62 cards)

1
Q

Name the types of megaloblastic anaemia and define the MCV level required.

A

Folate deficiency, pernicious anaemia, vitamin B12 deficiency
MCV>96fL

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2
Q

What is functional vitamin B12 deficiency?

A

Failure of intracellular transport of B12 by transcobalamin-2 can lead to functional B12 deficiency but with apparently normal serum levels. Neurological complications can occur.

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3
Q

What is folate and where is it absorbed?

A

Vitamin B9

Jejunum

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4
Q

What are the four causes of folate deficiency?

A

Dietary deficiency
Excessive requirements
Excessive urinary excretion
Antifolate drugs

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5
Q

Name three causes of excessive requirement of folate.

A

Pregnancy
Malignancy
Chronic inflammatory conditions

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6
Q

Name three anti-folate drugs.

A

Nitrofurantoin
Sulfasalazine
Methotrexate

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7
Q

What are megaloblasts?

A

Hypersegmented polymorphs with six or more lobes in the nucleus.

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8
Q

What is found on blood tests and blood films in folate deficiency anaemia?

A

MCV>96fL
Macrocytosis/megaloblasts
Serum folate<7nmol/L
<340nmol erythrocyte folate

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9
Q

What is the treatment of folic acid deficiency?

A

5mg folic acid OD for four months

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10
Q

Why is it important to check serum Vitamin B12 in patients with folate deficiency anaemia?

A

Folic acid may aggravate the neuropathy and precipitate subacute combined degeneration of the spinal cord.

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11
Q

Name three causes of microcytosis.

A

Pregnancy and the neonatal period
Alcohol excess and liver disease
Severe hypothyroidism

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12
Q

Where is Vitamin B12 absorbed and what does it require?

A

Terminal ileum

Requires intrinsic factor which is secreted by parietal cells

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13
Q

Give five causes of vitamin B12 deficiency.

A

Gastric causes e.g. gastrectomy, h.pylori infection
Vegan diet
Crohn’s disease
Long term PPI use

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14
Q

As well as typical symptoms of anaemia, what can Vitamin B12 deficiency present with?

A

Damage to peripheral nerves and posterior and lateral columns of spinal cord

Paraesthesiae
Numbness
Cognitive changes
Visual disturbances
Subacute combined degeneration of the cord
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15
Q

What are the signs of subacute combined degeneration of the cord?

A

Weakness, paraesthesiae, numbness of legs, arms, trunk –> Bilateral spastic paresis but loss of reflexes –> Decreased pressure, vibration, and touch sense –> Positive Babinski sign

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16
Q

How is Vitamin B12 deficiency diagnosed?

A
MCV>96fL
Macrocytosis/megaloblasts
Increased unconjugated bilirubin
Vitamin B12 low
Raised total homocysteine
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17
Q

What autoantibodies should be screened for in Vitamin B12 deficiency anaemia?

A

Intrinsic factor antibodies

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18
Q

What does it mean if the MCV is normal but Vitamin B12 is low?

A

There is a co-existing iron deficiency so there is a dimorphic blood film

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19
Q

What is the treatment of vitamin B12 deficiency anaemia?

A

Neuro involvement - 1mg Hydroxycobalamin IM 3x week for 2 weeks, then 1mg every 3 months
Neuro involvement - 1mg Hydroxycobalamin IM alternate days until no further improvement, then 1mg every 2 months

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20
Q

What are the indications for referral in B12 deficiency?

A

Haem - neuro symptoms, pregnancy, uncertain cause

Gastro - malabsorption is suspected.

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21
Q

Give four causes of iron deficiency anaemia.

A

GI/PV blood loss
Vegetarianism
PPI use
Tropical gut infestation

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22
Q

Give six signs of iron deficiency anaemia?

A
Pallor
Koilonychia
Angular cheilitis
Atrophic glossitis
Tachycardia
Flow murmur
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23
Q

What are the laboratory results in iron deficiency anaemia?

A

Hypochromic (low MCH) microcytic (low MCV) anaemia
Hb<12g/dl
Serum ferritin <12-15mcg

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24
Q

What does serum ferritin correlate with?

A

Total body iron stores

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25
What is seen on blood film in iron deficiency anaemia?
Anisocytosis (variety in size) | Poikilocytosis (abnormal shapes)
26
Which patients with iron deficiency should have upper/lower GI investigations?
All males | Postmenopausal females
27
What is the treatment of iron deficiency anaemia?
PO ferrous sulfate 200mg TD for 3 months
28
Name three side effects of ferrous sulfate.
Constipation/diarrhoea Black stools Nausea
29
What are three other causes of microcytic anaemia?
Haemoglobinopathies Sideroblastic anaemia Anaemia of chronic disease
30
What tests form part of a haemolytic screen?
``` Direct Coombs' test Bilirubin Reduced haptoglobin Reticulocytes Spherocytes LDH ```
31
By what mechanisms can haemolysis occur?
Intravascular (trauma etc) | Extravascular (removed because defective or Ig attached to surface)
32
Give six causes of haemolytic anaemia.
Red cell membrane - spherocytosis/elliptocytosis Haemoglobinopathies - sickle cell/thalassaemia Enzyme defects - G6PD, pyruvate kinase deficiency Autoimmune e.g. SLE Haemolytic disease of the newborn Malaria
33
Other than typical anaemia symptoms, name some S+S of haemolytic anaemia.
Gallstones - RUQ pain Haemoglobinuria Jaundice Splenomegaly
34
What is seen on the FBC in haemolytic anaemia?
Normocytic (Normal MCV) hypochromic (normal MCH) anaemia Low Hb Normal platelets, but thrombocytopenia can occur in SLE and CLL
35
What is the treatment of haemolytic anaemia?
Treat the cause e.g. splenectomy, iron therapy, transfusion therapy, corticosteroids if autoimmune
36
What are the two types of thalassaemia?
Alpha or beta
37
What are the subtypes of thalassemia?
Beta thalassemia minor, intermedia, and major Alpha thalassemia silent, trait, haemoglobin H disease, and major (1, 2, 3, and 4 alpha HbA alleles affected respectively)
38
What is the prognosis of alpha thalassemia homozygous?
Death in utero: hydrops fetalis
39
Which type of thalassemia has the more severe symptoms?
Alpha > Beta
40
What is the gold standard of diagnosis in thalassemia?
Haemoglobin electrophoresis: HbA2>3.5%
41
What other investigations are required in suspected thalassemia?
Low Hb, low MCH, low MCV (microcytic hypochromic) | Raised serum iron and ferritin
42
What are the symptoms of thalassemia trait?
Asymptomatic
43
What are the signs of heterozygous thalassemia?
Anaemia Splenomegaly Bone changes (frontal bossing, prominent facial bones, dental malocclusion)
44
What are the signs of homozygous beta thalassemia?
Severe haemolytic anaemia | Hepatosplenomegaly
45
What is the management of thalassemia?
Intermedia: transfusion if pregnant/ill Major: chronic transfusion dependency
46
What is the pathophysiology of sickle cell disease?
Cells have a reduced deformability and are easily destroyed | There is occlusion of microcirculation and chronic haemolytic anaemia
47
What are the main types of sickle cell disease?
HbSS/Sickle cell anaemia - homozygous HbSC/Sickle cell trait - heterozygous (40% HbS) Sickle beta plus thalassemia Sickle HbD
48
What is the inheritance pattern of sickle cell disease and thalassemia?
Autosomal recessive
49
How does sickle cell trait present?
Usually asymptomatic Protects against malaria Occasionally haematuria, decreased ability to concentrate urine, renal papillary necrosis, splenic infarction, exertional rhabdomyolysis
50
When does sickle cell anaemia present?
Between 3 and 6 months of age when HbF levels are falling
51
Give five symptoms/presentations of sickle cell anaemia.
Acute splenic sequestration Acute chest syndrome Increased susceptibility to encapsulated bacteria such as pneumococcus Splenomegaly but recurrent splenic infarcts cause autosplenectomy Anaemia, jaundice, lethargy, growth restriction
52
How is sickle cell anaemia diagnosed?
Low Hb, high reticulocytes Blood film shows sickled erythrocytes Haemoglobin electrophoresis: no HbA, 80% HbSS, 20% HbF
53
What is the treatment of sickle cell anaemia?
Hydroxycarbamide Penicillin prophylaxis Avoid precipitating crises
54
What is a treatment of major haemorrhage (except surgery)?
Tranexamic acid | IV bolus followed by slow infusion
55
How long before an operation should the COCP be stopped for?
4 weeks
56
How is anaemia of chronic disease diagnosed?
Increased ferritin Decreased total iron binding capacity (TIBC) Normo/microcytic
57
In blood transfusions, what is a non-haemolytic febrile transfusion reaction?
Mild fever due to an acute reaction to plasma proteins If no other symptoms, continue transfusion, treat with paracetamol, and increase observations.
58
What does phenytoin cause?
Folate deficiency with microcytosis or just macrocytosis
59
What is glucose 6-phosphate dehydrogenase deficiency and what are the triggers for symptoms?
X-linked red cell enzymopathy renders RBC vulnerable to oxidative stress and haemolysis under certain conditions (asymptomatic until triggers) Triggers: - Fava beans - Aspirin - Infection - Anti-malarials
60
What are the causes of sickle crises in sickle cell disease?
HbS has an increased affinity for oxygen than adult haemoglobin which results in crises in conditions of infection, cold, stress, and hypoxia.
61
Why are sickle cell patients at increased risk of infection with encapsulated bacteria?
Autosplenectomy
62
What are the characteristics of aplastic anaemia?
Pancytopenia and lack of reticulocytes | Hypocellular bone marrow with increased fat spaces