Anaemia Flashcards
1
Q
Definition anaemia
A
Reduction in red cells or their haemoglobin content
Or increased plasma volume
2
Q
Example of situation where dilutional anaemia can occur
A
Pregnancy
3
Q
Name some substances required for making red cells
A
Iron
B12
Folic acid
Erythropoietin
4
Q
3 broad types anaemia
A
Hypochromic Microcytic anaemia
Normochromic Normocytic
Macroytic
5
Q
Symptoms in anaemia
A
Fatigue Dyspnoea Faintness Pallor Oedema Chest pain Palpitations Headache Tinnitus Anorexia
6
Q
Initial Rx for microcytic anaemia
A
Serum ferritin
7
Q
Initial Ix for normochromic normocytic anaemia
A
Reticulocyte count
8
Q
Initial Ix for Macrocytic anaemia
A
Do B12/folate
9
Q
Which is the commonest type of anaemia
A
Hypochromic microcytic
10
Q
What is the main cause of hypochromic microcytic anaemia
A
Iron deficiency
11
Q
Hypochromic microcytic anaemia with low ferritin what is the Dx
A
Iron deficiency anaemia
12
Q
Hypochromic microcytic anaemia with normal ferritin what are potential Dx
A
Thalassemia
Sideroblastic anaemia
30% of secondary anaemia
13
Q
Which is the commonest anaemia worldwide
A
Iron deficiency
14
Q
Causes of iron deficiency anaemia
A
Blood loss:
Menorrhagia
GI bleeding
Poor diet
Malabsorption:
Coeliac disease
Gastrectomy
15
Q
Signs of iron deficiency
A
Koilonychia (spoon shaped nails_
Angular cheilitiis (around mouth)
Atrophic tongue
16
Q
Ix for iron deficiency anaemia
A
Blood film
Serum ferritin low
Finding cause:
Coeliac serology
Endoscopy
Colonoscopy
17
Q
Rx for iron deficiency anaemia
A
Oral iron
IV if intolerable to oral
18
Q
SE of oral iron
A
Nausea Abdo. discomfort Diarrhoea Constipation Black stools
19
Q
What is thalassaemia
A
Reduced or absent globin chain production
Type of haemoglobinopathy
20
Q
How many alpha chains are there
A
4
21
Q
How many beta chains are there
A
2
22
Q
Describe features of (α0/α0 ):
A
Incompatible with life
Death in utero
23
Q
Features of (–/-a)
A
Moderate anaemia Features of haemolysis Hepatosplenomegaly Leg ulcers Jaundice
24
Q
Features of (–/aa)
A
Asymptomatic carrier state
25
Features of (-a/aa)
Clinical state is normal
26
Describe thalassaemia beta minor
Heterozygous state
| Carirer state
27
Describe Beta thalassaemia intermedia
Intermediate state
Moderate anaemia
but not requiring transfusion
28
Describe beta thalassaemia major
Homozygous state
Sig. abnormality in both beta globin chains
Transfusion dependent
29
Which beta thalassaemia is transfusion dependent
Major
30
Which beta thalassaemia has anaemia not relying on transfusions
Intermedia
31
Signs of beta minor
Usually asymptomatic
Mild well tolerated anaemia
May worsen during pregnancy
32
Signs of beta intermedia
Splenomegaly
| Moderate anaemia
33
Signs of beta major
```
Severe anaemia
Failure to thrive
Presents early
Hepatosplenomegaly
Skull bossing
‘Hair on end’ sign
Due to marrow activity
```
34
Ix for thalassaemia
```
FBC
MCV
Iron
HbA2
HbF
Hb electrophoresis
```
35
Describe ferritin level in thalassaemia
Normal or increased
36
What is iron chelation therapy
Drug to remove excess iron to prevent overloading
37
Examples of iron chelation drugs
S/C - desferrioxamine infusions
| Oral – Deferasirox
38
Rx for beta major
Chronic transfusion
Iron chelation therapy
Splenectomy
Hormonal replacement or Rx for endocrine complications
39
Rx beta intermedia
Required treatment for anaemia
| NOT transfusions
40
Rx for beta minor
Relatively asymptomatic
No Rx
May require iron in pregnancy
41
Normochromic Normocytic anaemia with increased reticulocyte count what are the Ddx
Acute blood loss
| Haemolysis
42
Normochromic Normocytic anaemia with normal/decreased reticulocyte count what are the Ddx
70% secondary anaemia
Hypoplasia
Marrow infiltration
43
What % of secondary anaemia is normochromic and hypochromic
70% normo
| 30% hypo
44
What is haemolytic anaemia
Accelerated red cell destruction before their normal lifespan
45
What are the effects of increased RBC destruction
Increased bilirubin
| Increased faecal and urinary urobilinogen
46
What is the bone marrow compensation for haemolysis
Reticulocytosis
| Hence increased reticulocytes
47
Is intravascular haemolysis (in the blood vessels) mainly immune or non-immune
Non-immune
48
Is extravascular haemolysis (elsewhere in the body) usually immune or non immune
Immune
49
Conditions that can cause congenital haemolysis
Hereditary spherocytosis (HS)
Enzyme deficiency (G6PD deficiency)
Haemoglobinopathy (HbSS)
E.g Thalassaemia, Sickle Cell
50
Non-immune mediated causes of haemolysis
Underlying malignancy
Result of hypersplenism (e.g portal hypertension)
Lead poisoning
Mechanical e.g artificial valve
Severe infection (DIC, PET, HUS)
51
Immune mediated cause of haemolytic anaemia
Auto-immune haemolytic anaemia
52
Tests to Dx haemolysis
```
FBC
Blood film
Reticulocyte count
Serum bilirubin
LDH
Serum haptoglobin
```
Coombs test Urine for haemosiderin/
urobilinogen
Indirect Coombs
53
Rx for haemolysis
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
Describe Direct Antiglobulin test
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
What is autoimmune haemolytic anaemia
Type of anaemia
| Mediated by autoantibodies causing mainly extravascular haemolysis and spherocytosis
56
Secondary causes of warm AIHA
CLL
Lymphoma
Drugs
Autoimmune disease
57
Secondary causes of cold AIHA
Infection
CHAD
Lymphoma
58
Cause of AIHA
mainly idiopathic
59
Compared warm and cold AIHA
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
How is AIHA classified
Warm and cold
| According to optimal binding to temperature to RBC
61
Rx for warm AIHA
Steroids
Immunosuppression
+/- splenectomy
62
Rx for cold AIHA
Keep warm
63
What is the 2nd most common anaemia
Secondary
64
Describe 2ndry anaemia
When there is an identifiable underlying disease
65
Describe ferritin in secondary anaemia
Usually elevated
66
What is hereditary spherocytosis
Type of congenital anaemia
| Caused by defective RBC membrane
67
Inheritance of hereditary spherocytosis
Most common form
| Autosomal dominant
68
Which proteins are defective in hereditary spherocytosis
```
5 structural proteins:
Ankyrin
Alpha spectrin
Beta spectrin
Band 3
Protein 4.2
```
69
Mechanism of haemolysis in hereditary spherocytosis
RBC are defective in shape
| Recognised defective and removed from the circulation by the RE system in the spleen
70
Shape of RBC in hereditary spherocytosis
Spherical
71
Rx for hereditary spherocytosis
As congenital usually compensation occurs
Folic acid
Transfusion
Splenectomy
72
Name 3 other rare RBC membrane disorders
Hereditary Elliptocytosis
Hereditary Pyropoilkilocytosis
South East Asian Ocalocytosis
73
What is the chief RBC enzyme disorder
G6PD deficiency
74
Hereditary pattern of G6PD deficiency
X linked
75
Blood film appearance in G6PD deficiency
Bite cells
76
Clinical presentation of G6PD deficiency
Neonatal jaundice
Drug, broad bean or infection precipitated jaundice and anaemia
Splenomegaly
Pigment gallstones
77
Haemolysis triggers in G6PD deficiency
Infection
| Drugs
78
Which drugs can trigger haemolysis in G6PD deficiency
Anti-malarials e.g Primaquine
Anti-bacterials e.g Nitrafurantoin
Analgesics e.g Aspirin
Sulphonamides and Sulphones
79
Ix for G6PD deficiency
Enzme assay
80
Rx for G6PD deficiency
Avoid precipitants
| Transfuse if severe
81
Name another rare RBC enzyme deficiency
Pyruvate Kinase Deficiency
82
Cause of megaloblastic anaemia
B12 deficiency
| Folate deficiency
83
Cause of non-megaloblastic anaemia
Myelodysplasia
Marrow infiltration
Drugs
84
Initial Rx for macrocytic anaemia
B12/Folate assay
85
What does vitamin B12 bind to in the stomach
Intrinsic factor
86
Which cells produce intrinsic factor
Parietal cells
87
Where does final absorption of B12 take place
Terminal ileum
88
Causes of Vitamin B12 deficiency
Pernicious anaemia (autoimmune)
Gastrectomy (removal of stomach hence removal of B12 producing parietal cells)
Poor dietary intake
Gastric/ileal disease
89
Signs of Vitamin B12 deficiency
Anaemia
Neurological symptoms:
Paraesthesia
Peripheral neuropathy
Lemon tinge skin
Glossitis
90
Which system can Vit B12 deficiency have serious consequences on
Neurological
91
Describe pernicious anaemia
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
Associations of pernicious aneamia
Other autoimmune disease
Thyroid
Addison’s
Hypoparathyroidism
93
Ix for pernicious anaemia
Antibodies against
Intrinsic factor (Dx)
Gastric parietal cells (less specific)
Serum B12
Hb
WCC
MCV
94
Rx for pernicious anaemia
IM B12 injections
Initial loading dose
Then every 3/12
95
What is 3x more common in pernicious anaemia
Stomach carcinoma
96
Another name for Folate acid
Vitamin B9
97
Causes of folate deficiency
```
Dietary
Increased requirement (haemolysis, pregnancy)
GI pathology (e.g Coeliac)
```
98
Rx for folate deficiency
Oral folate replacement
