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Haematology and Immunology > Anaemia > Flashcards

Anaemia Flashcards

1
Q

Definition anaemia

A

Reduction in red cells or their haemoglobin content

Or increased plasma volume

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2
Q

Example of situation where dilutional anaemia can occur

A

Pregnancy

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3
Q

Name some substances required for making red cells

A

Iron
B12
Folic acid
Erythropoietin

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4
Q

3 broad types anaemia

A

Hypochromic Microcytic anaemia
Normochromic Normocytic
Macroytic

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5
Q

Symptoms in anaemia

A 
Fatigue 
Dyspnoea 
Faintness 
Pallor 
Oedema 
Chest pain 
Palpitations 
Headache 
Tinnitus 
Anorexia
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6
Q

Initial Rx for microcytic anaemia

A

Serum ferritin

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7
Q

Initial Ix for normochromic normocytic anaemia

A

Reticulocyte count

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8
Q

Initial Ix for Macrocytic anaemia

A

Do B12/folate

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9
Q

Which is the commonest type of anaemia

A

Hypochromic microcytic

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10
Q

What is the main cause of hypochromic microcytic anaemia

A

Iron deficiency

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11
Q

Hypochromic microcytic anaemia with low ferritin what is the Dx

A

Iron deficiency anaemia

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12
Q

Hypochromic microcytic anaemia with normal ferritin what are potential Dx

A

Thalassemia
Sideroblastic anaemia
30% of secondary anaemia

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13
Q

Which is the commonest anaemia worldwide

A

Iron deficiency

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14
Q

Causes of iron deficiency anaemia

A

Blood loss:
Menorrhagia
GI bleeding

Poor diet

Malabsorption:
Coeliac disease
Gastrectomy

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15
Q

Signs of iron deficiency

A

Koilonychia (spoon shaped nails_
Angular cheilitiis (around mouth)
Atrophic tongue

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16
Q

Ix for iron deficiency anaemia

A

Blood film
Serum ferritin low

Finding cause:
Coeliac serology
Endoscopy
Colonoscopy

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17
Q

Rx for iron deficiency anaemia

A

Oral iron

IV if intolerable to oral

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18
Q

SE of oral iron

A 
Nausea 
Abdo. discomfort 
Diarrhoea 
Constipation 
Black stools
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19
Q

What is thalassaemia

A

Reduced or absent globin chain production

Type of haemoglobinopathy

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20
Q

How many alpha chains are there

A

4

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21
Q

How many beta chains are there

A

2

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22
Q

Describe features of (α0/α0 ):

A

Incompatible with life

Death in utero

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23
Q

Features of (–/-a)

A 
Moderate anaemia 
Features of haemolysis 
Hepatosplenomegaly 
Leg ulcers
Jaundice
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24
Q

Features of (–/aa)

A

Asymptomatic carrier state

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25
Q

Features of (-a/aa)

A

Clinical state is normal

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26
Q

Describe thalassaemia beta minor

A

Heterozygous state

Carirer state

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27
Q

Describe Beta thalassaemia intermedia

A

Intermediate state
Moderate anaemia
but not requiring transfusion

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28
Q

Describe beta thalassaemia major

A

Homozygous state
Sig. abnormality in both beta globin chains
Transfusion dependent

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29
Q

Which beta thalassaemia is transfusion dependent

A

Major

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30
Q

Which beta thalassaemia has anaemia not relying on transfusions

A

Intermedia

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31
Q

Signs of beta minor

A

Usually asymptomatic
Mild well tolerated anaemia
May worsen during pregnancy

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32
Q

Signs of beta intermedia

A

Splenomegaly

Moderate anaemia

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33
Q

Signs of beta major

A 
Severe anaemia
Failure to thrive 
Presents early 
Hepatosplenomegaly 
Skull bossing 
‘Hair on end’ sign 
Due to  marrow activity
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34
Q

Ix for thalassaemia

A 
FBC 
MCV 
Iron 
HbA2
HbF 
Hb electrophoresis
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35
Q

Describe ferritin level in thalassaemia

A

Normal or increased

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36
Q

What is iron chelation therapy

A

Drug to remove excess iron to prevent overloading

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37
Q

Examples of iron chelation drugs

A

S/C - desferrioxamine infusions

Oral – Deferasirox

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38
Q

Rx for beta major

A

Chronic transfusion
Iron chelation therapy
Splenectomy
Hormonal replacement or Rx for endocrine complications

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39
Q

Rx beta intermedia

A

Required treatment for anaemia

NOT transfusions

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40
Q

Rx for beta minor

A

Relatively asymptomatic
No Rx
May require iron in pregnancy

41
Q

Normochromic Normocytic anaemia with increased reticulocyte count what are the Ddx

A

Acute blood loss

Haemolysis

42
Q

Normochromic Normocytic anaemia with normal/decreased reticulocyte count what are the Ddx

A

70% secondary anaemia
Hypoplasia
Marrow infiltration

43
Q

What % of secondary anaemia is normochromic and hypochromic

A

70% normo

30% hypo

44
Q

What is haemolytic anaemia

A

Accelerated red cell destruction before their normal lifespan

45
Q

What are the effects of increased RBC destruction

A

Increased bilirubin

Increased faecal and urinary urobilinogen

46
Q

What is the bone marrow compensation for haemolysis

A

Reticulocytosis

Hence increased reticulocytes

47
Q

Is intravascular haemolysis (in the blood vessels) mainly immune or non-immune

A

Non-immune

48
Q

Is extravascular haemolysis (elsewhere in the body) usually immune or non immune

A

Immune

49
Q

Conditions that can cause congenital haemolysis

A

Hereditary spherocytosis (HS)
Enzyme deficiency (G6PD deficiency)
Haemoglobinopathy (HbSS)
E.g Thalassaemia, Sickle Cell

50
Q

Non-immune mediated causes of haemolysis

A

Underlying malignancy

Result of hypersplenism (e.g portal hypertension)

Lead poisoning

Mechanical e.g artificial valve

Severe infection (DIC, PET, HUS)

51
Q

Immune mediated cause of haemolytic anaemia

A

Auto-immune haemolytic anaemia

52
Q

Tests to Dx haemolysis

A 
FBC
Blood film 
Reticulocyte count 
Serum bilirubin 
LDH 
Serum haptoglobin

Coombs test Urine for haemosiderin/
urobilinogen

Indirect Coombs

53
Q

Rx for haemolysis

A

Support marrow function:
Folic acid

Correct cause:

Autoimmune:
Steroids
Treat trigger e.g CLL, lymphoma

Remover site of red cell destruction:
Splenectomy

Consider transfusion

54
Q

Describe Direct Antiglobulin test

A

Test to Dx auto-immune anaemia
Detects antibody or complement on red cell membrane
Reagent contains either:
Anti-human IgG
Anti-complement
Reagent binds to Ab (or complement) on red cell surface and causes agglutinatin in vitro
Implies immune basis for haemolysis

DAGT +ve = immune mediated
DAGT –ve = non-immune related

55
Q

What is autoimmune haemolytic anaemia

A

Type of anaemia

Mediated by autoantibodies causing mainly extravascular haemolysis and spherocytosis

56
Q

Secondary causes of warm AIHA

A

CLL
Lymphoma
Drugs
Autoimmune disease

57
Q

Secondary causes of cold AIHA

A

Infection
CHAD
Lymphoma

58
Q

Cause of AIHA

A

mainly idiopathic

59
Q

Compared warm and cold AIHA

A

Warm AIHA
IgG mediated
Bind at 370 body t.

Cold AIHA:
IgM mediated
Bind at temp (<4C0)
Activating cell-surface complement 
Often made worse by cold e.g Raynauds
60
Q

How is AIHA classified

A

Warm and cold

According to optimal binding to temperature to RBC

61
Q

Rx for warm AIHA

A

Steroids
Immunosuppression
+/- splenectomy

62
Q

Rx for cold AIHA

A

Keep warm

63
Q

What is the 2nd most common anaemia

A

Secondary

64
Q

Describe 2ndry anaemia

A

When there is an identifiable underlying disease

65
Q

Describe ferritin in secondary anaemia

A

Usually elevated

66
Q

What is hereditary spherocytosis

A

Type of congenital anaemia

Caused by defective RBC membrane

67
Q

Inheritance of hereditary spherocytosis

A

Most common form

Autosomal dominant

68
Q

Which proteins are defective in hereditary spherocytosis

A 
5 structural proteins:
Ankyrin 
Alpha spectrin 
Beta spectrin 
Band 3 
Protein 4.2
69
Q

Mechanism of haemolysis in hereditary spherocytosis

A

RBC are defective in shape

Recognised defective and removed from the circulation by the RE system in the spleen

70
Q

Shape of RBC in hereditary spherocytosis

A

Spherical

71
Q

Rx for hereditary spherocytosis

A

As congenital usually compensation occurs
Folic acid
Transfusion
Splenectomy

72
Q

Name 3 other rare RBC membrane disorders

A

Hereditary Elliptocytosis
Hereditary Pyropoilkilocytosis
South East Asian Ocalocytosis

73
Q

What is the chief RBC enzyme disorder

A

G6PD deficiency

74
Q

Hereditary pattern of G6PD deficiency

A

X linked

75
Q

Blood film appearance in G6PD deficiency

A

Bite cells

76
Q

Clinical presentation of G6PD deficiency

A

Neonatal jaundice
Drug, broad bean or infection precipitated jaundice and anaemia
Splenomegaly
Pigment gallstones

77
Q

Haemolysis triggers in G6PD deficiency

A

Infection

Drugs

78
Q

Which drugs can trigger haemolysis in G6PD deficiency

A

Anti-malarials e.g Primaquine
Anti-bacterials e.g Nitrafurantoin
Analgesics e.g Aspirin
Sulphonamides and Sulphones

79
Q

Ix for G6PD deficiency

A

Enzme assay

80
Q

Rx for G6PD deficiency

A

Avoid precipitants

Transfuse if severe

81
Q

Name another rare RBC enzyme deficiency

A

Pyruvate Kinase Deficiency

82
Q

Cause of megaloblastic anaemia

A

B12 deficiency

Folate deficiency

83
Q

Cause of non-megaloblastic anaemia

A

Myelodysplasia
Marrow infiltration
Drugs

84
Q

Initial Rx for macrocytic anaemia

A

B12/Folate assay

85
Q

What does vitamin B12 bind to in the stomach

A

Intrinsic factor

86
Q

Which cells produce intrinsic factor

A

Parietal cells

87
Q

Where does final absorption of B12 take place

A

Terminal ileum

88
Q

Causes of Vitamin B12 deficiency

A

Pernicious anaemia (autoimmune)
Gastrectomy (removal of stomach hence removal of B12 producing parietal cells)
Poor dietary intake
Gastric/ileal disease

89
Q

Signs of Vitamin B12 deficiency

A

Anaemia
Neurological symptoms:
Paraesthesia
Peripheral neuropathy

Lemon tinge skin
Glossitis

90
Q

Which system can Vit B12 deficiency have serious consequences on

A

Neurological

91
Q

Describe pernicious anaemia

A

Autoimmune disease Atrophic gastritis leads to lack of intrinsic factor secretion from parietal cells in the stomach
Dietary B12 therefore remains unbound
And not absorbed at the terminal ileum

92
Q

Associations of pernicious aneamia

A

Other autoimmune disease
Thyroid
Addison’s
Hypoparathyroidism

93
Q

Ix for pernicious anaemia

A

Antibodies against
Intrinsic factor (Dx)
Gastric parietal cells (less specific)

Serum B12
Hb
WCC
MCV

94
Q

Rx for pernicious anaemia

A

IM B12 injections
Initial loading dose
Then every 3/12

95
Q

What is 3x more common in pernicious anaemia

A

Stomach carcinoma

96
Q

Another name for Folate acid

A

Vitamin B9

97
Q

Causes of folate deficiency

A 
Dietary 
Increased requirement (haemolysis, pregnancy)
GI pathology (e.g Coeliac)
98
Q

Rx for folate deficiency

A

Oral folate replacement

Haematology and Immunology (7 decks)

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