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Phase 3B > Haematology - anaemia > Flashcards

Haematology - anaemia Flashcards

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
Q

What is seen on blood film in iron deficiency anaemia?

A

Anisocytosis (variety in size)

Poikilocytosis (abnormal shapes)

26
Q

Which patients with iron deficiency should have upper/lower GI investigations?

A

All males

Postmenopausal females

27
Q

What is the treatment of iron deficiency anaemia?

A

PO ferrous sulfate 200mg TD for 3 months

28
Q

Name three side effects of ferrous sulfate.

A

Constipation/diarrhoea
Black stools
Nausea

29
Q

What are three other causes of microcytic anaemia?

A

Haemoglobinopathies
Sideroblastic anaemia
Anaemia of chronic disease

30
Q

What tests form part of a haemolytic screen?

A 
Direct Coombs' test
Bilirubin
Reduced haptoglobin
Reticulocytes
Spherocytes
LDH
31
Q

By what mechanisms can haemolysis occur?

A

Intravascular (trauma etc)

Extravascular (removed because defective or Ig attached to surface)

32
Q

Give six causes of haemolytic anaemia.

A

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
Q

Other than typical anaemia symptoms, name some S+S of haemolytic anaemia.

A

Gallstones - RUQ pain
Haemoglobinuria
Jaundice
Splenomegaly

34
Q

What is seen on the FBC in haemolytic anaemia?

A

Normocytic (Normal MCV) hypochromic (normal MCH) anaemia
Low Hb
Normal platelets, but thrombocytopenia can occur in SLE and CLL

35
Q

What is the treatment of haemolytic anaemia?

A

Treat the cause e.g. splenectomy, iron therapy, transfusion therapy, corticosteroids if autoimmune

36
Q

What are the two types of thalassaemia?

A

Alpha or beta

37
Q

What are the subtypes of thalassemia?

A

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
Q

What is the prognosis of alpha thalassemia homozygous?

A

Death in utero: hydrops fetalis

39
Q

Which type of thalassemia has the more severe symptoms?

A

Alpha > Beta

40
Q

What is the gold standard of diagnosis in thalassemia?

A

Haemoglobin electrophoresis: HbA2>3.5%

41
Q

What other investigations are required in suspected thalassemia?

A

Low Hb, low MCH, low MCV (microcytic hypochromic)

Raised serum iron and ferritin

42
Q

What are the symptoms of thalassemia trait?

A

Asymptomatic

43
Q

What are the signs of heterozygous thalassemia?

A

Anaemia
Splenomegaly
Bone changes (frontal bossing, prominent facial bones, dental malocclusion)

44
Q

What are the signs of homozygous beta thalassemia?

A

Severe haemolytic anaemia

Hepatosplenomegaly

45
Q

What is the management of thalassemia?

A

Intermedia: transfusion if pregnant/ill
Major: chronic transfusion dependency

46
Q

What is the pathophysiology of sickle cell disease?

A

Cells have a reduced deformability and are easily destroyed

There is occlusion of microcirculation and chronic haemolytic anaemia

47
Q

What are the main types of sickle cell disease?

A

HbSS/Sickle cell anaemia - homozygous
HbSC/Sickle cell trait - heterozygous (40% HbS)
Sickle beta plus thalassemia
Sickle HbD

48
Q

What is the inheritance pattern of sickle cell disease and thalassemia?

A

Autosomal recessive

49
Q

How does sickle cell trait present?

A

Usually asymptomatic
Protects against malaria
Occasionally haematuria, decreased ability to concentrate urine, renal papillary necrosis, splenic infarction, exertional rhabdomyolysis

50
Q

When does sickle cell anaemia present?

A

Between 3 and 6 months of age when HbF levels are falling

51
Q

Give five symptoms/presentations of sickle cell anaemia.

A

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
Q

How is sickle cell anaemia diagnosed?

A

Low Hb, high reticulocytes
Blood film shows sickled erythrocytes
Haemoglobin electrophoresis: no HbA, 80% HbSS, 20% HbF

53
Q

What is the treatment of sickle cell anaemia?

A

Hydroxycarbamide
Penicillin prophylaxis
Avoid precipitating crises

54
Q

What is a treatment of major haemorrhage (except surgery)?

A

Tranexamic acid

IV bolus followed by slow infusion

55
Q

How long before an operation should the COCP be stopped for?

A

4 weeks

56
Q

How is anaemia of chronic disease diagnosed?

A

Increased ferritin
Decreased total iron binding capacity (TIBC)
Normo/microcytic

57
Q

In blood transfusions, what is a non-haemolytic febrile transfusion reaction?

A

Mild fever due to an acute reaction to plasma proteins

If no other symptoms, continue transfusion, treat with paracetamol, and increase observations.

58
Q

What does phenytoin cause?

A

Folate deficiency with microcytosis

or just macrocytosis

59
Q

What is glucose 6-phosphate dehydrogenase deficiency and what are the triggers for symptoms?

A

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
Q

What are the causes of sickle crises in sickle cell disease?

A

HbS has an increased affinity for oxygen than adult haemoglobin which results in crises in conditions of infection, cold, stress, and hypoxia.

61
Q

Why are sickle cell patients at increased risk of infection with encapsulated bacteria?

A

Autosplenectomy

62
Q

What are the characteristics of aplastic anaemia?

A

Pancytopenia and lack of reticulocytes

Hypocellular bone marrow with increased fat spaces

Phase 3B (20 decks)

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