Anaemia Flashcards
(124 cards)
1
Q
Anaemia
A
Reduced total red cell mass
2
Q
What is the normal response to anaemia
A
Reticulocytosis
3
Q
Reticulocytosis
A
The increase of production of immature red blood cells
4
Q
When is a blood film described as polychromatic
A
When there are too many reticulocytes
5
Q
Why do reticulocytes stain blue/purple
A
Still have remnants of RNA
6
Q
Symptoms of anaemia (5)
A
Breathlessness
Fatigue
Headaches
Palpitations
Faintness
7
Q
Investigations for anaemia (3)
A
FBC
blood film
Reticulocyte count
8
Q
What does haematocrit measure
A
Ratio of the whole blood that is red cells
9
Q
Microcytic anaemia
A
Anaemia caused by deficient haemoglobin synthesis
10
Q
Causes of Microcytic anaemia
A
TAILS
Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia
11
Q
What are the 3 general causes of iron deficiency
A
Insufficient intake
Loosing too much
Not absorbing enough
12
Q
What can cause you to not absorb enough iron
A
Coeliac disease
13
Q
What happens to % saturation of transferrin in hereditary haemochromatosis
A
Increases
14
Q
Where is iron mostly stored in the body
A
Liver
15
Q
Renal anaemia
A
Anaemia of chronic disease due to failure of erythropoietin production
16
Q
Why do we get anaemia of chronic disease
A
Protective mechanism to reduce the supply of iron to pathogens
17
Q
Pathophysiology of anaemia of chronic disease (5)
A
- inflammatory cytokines result in increased transcription of ferritin mRNA
- ferritin synthesis increases
- inflammatory cytokines also causes an increase plasma hepcidin
- blocks ferroportin-mediated release of iron
- impaired iron supply to marrow erythroblasts
18
Q
Clinical features of long standing iron deficiency (6)
A
- brittle nails
- koilonychia
- atrophy of tongue papillae
- angular stomatitis
- brittle hair
- dysphasia and glottitis
19
Q
Transferrin levels in iron deficiency anaemia
A
Normal or increased
20
Q
% transferrin saturation in iron deficiency anaemia
A
Reduced
21
Q
MCV in iron deficiency anaemia
A
Reduced
22
Q
Serum iron in Anaemia of chronic disease
A
Reduced
23
Q
Transferrin levels in Anaemia of chronic disease
A
Normal or reduced
24
Q
% transferrin saturation in Anaemia of chronic disease
A
Reduced
25
Ferritin levels in Anaemia of chronic disease
Normal or increased
26
MCV in Anaemia of chronic disease
Normal (or can be reduced)
27
When is oral iron recommended to be taken
30 mins before a meal or 2 hours before other drugs
28
What should you avoid taking with oral iron
Calcium, antacids, caffeine and high fibre foods
29
Side effects of oral iron
Constipation, N+V , abdominal pains and dark stools
30
When is IV iron recommended
ONLY if oral is unsuccessful
31
Megaloblastic anaemia
Presence of erythroblasts in the bone marrow with delayed nuclear maturation because of defective DNA synthesis
32
What are the main 2 causes of megaloblastic anaemia
Folate and B12 deficiency
33
Causes of B12 deficiency (7)
- low intake
- pernicious anaemia
- PPI and H2 antagonists
- bypass surgery
- chronic pancreatitis
- coeliac disease
- chronic
34
Causes of folate deficiency (6)
- low intake
- coeliac
- chrons
- anticonvulsants
- pregnancy
- malignancy
35
Megaloblasts
Abnormally large red cell precursor with an immature nucleus
36
Where are megaloblasts normally found
Bone marrow
37
State 2 biochemical reactions in which folate and B12 are needed
DNA synthesis and nuclear maturation
DNA modification and gene activity
38
Where is B12 absorbed
Ileum
39
Where is folate absorbed
Duodenum and jejunum
40
What extra complications can B12 deficiency cause
Neurological
41
Blood film findings for megaloblastic anaemia (2)
Macrovalocytes
Hypersegmented neutrophils
42
What auto-antibodies might you check for in megaloblastic anaemia (2)
Anti gastric parietal cell
Anti intrinsic factor
43
Management of pernicious anaemia
B12 injections for life
44
Management of folate deficiency anaemia
Folic acid tablets
45
State 4 causes of non-megaloblastic macrocytosis
- alcohol
- liver disease
- hypothyroidism
- marrow failure
46
What happens in spurious macrocytosis
The volume of mature red cells are normal but MCV is measured as high as
47
State 2 causes of spurious macrocytosis
Reticulocytosis in response to acute blood loss or haemolysis
Cold-agglutinins
48
What is thalassaemia
Disorder which causes reduced globin chain synthesis
49
What kind of inheritance is seen in thalassaemia
Autosomal recessive
50
Alpha thalassaemia
Defects in alpha globin chains
51
Beta thalassaemia
Defects in beta globin chains
52
What causes alpha thalassaemia
Deletion of one or both alpha genes on chromosome 16
53
What causes a patient to have A thalassaemia trait
Missing one or two alpha genes
54
Clinical presentation of someone with a thalassaemia trait
Microcytic hypochromic red cells with mild anaemia
55
How do you differentiate between iron deficiency anaemia and a thalassaemia trait
Low ferritin in iron deficiency anaemia
56
What causes HbH disease
Only one alpha gene left
57
What is a consequence of HbH disease
Excess b chains form tetrameres
58
Where is HbH disease mostly found
SE Asia
59
State 2 key signs of HbH disease
Jaundice and splenomegaly
60
What causes Hb Bart’s hydrops fetalis
No functional alpha genes
61
Consequence of Hb Bart’s hydrops fetalis
No alpha chains so tetrameres of Hb Bart’s and HbH are produced
62
Clinical features of Hb Bart’s hydrops fetalis (5)
- profound anaemia
- cardiac failure
- growth retardation
- severe hepatosplenomegaly
- skeletal and cardio abnormalities
63
What causes beta thalassaemia
Point mutation on chromosome 11
64
Thalassaemia intermedia
Beta genes are present but there’s a mutation
65
Thalassaemia major
Beta globin genes are missing
66
When do patients with b thalassaemia major tend to present
6-24 months
67
Presentation of a patient with b thalassaemia major (4)
- pallor
- failure to thrive
- hepatosplenomegaly
- skeletal changes
68
Management of b thalassaemia major
Regular transfusion
69
What kind of anaemia does thalassaemia present with
Microcytic hypochromic
70
Hb analysis findings of a patient with b thalassaemia major
Mainly HbF , NO HbA
71
What is diagnostic for b thalassaemia trait
Raised HbA2
72
How do we quantify haemoglobin present in the body (2)
- electrophoresis
- high performance liquid chromatography
73
Complication of managing thalassaemia
Iron overload
74
Haemolysis
Premature red cell destruction
75
Signs of G6PD deficiency on a blood film
Heinz bodies
76
Sign of membrane damage on a blood film
Spherocytes
77
What do Sickle syndromes affect
The structure of haemoglobin
78
What kind of mutation is seen is sickle syndromes
Point mutation on codon 6 of the b globin gene
79
What is the consequence of the mutation in sickle syndromes
Glutamine is substituted to valine producing bS
80
What happens if a patient with sickle syndrome becomes hypoxic
Red blood cells become distorted damaging their membrane
81
Describe haemoglobin in sickle cell trait
One normal and one abnormal b gene
82
Is someone sickle cell trait usually symptomatic or asymptomatic
Asymptomatic
83
When may a patient with sickle cell trait present
Severe hypoxia : high altitude, under anaesthesia
84
Blood film in a patient with sickle cell trait
Normal
85
What is the proportion of HbS in sickle cell trait
<50%
86
Genetic presentation of sickle cell anaemia
2 abnormal b genes
87
Proportion of HbS in patients with sickle cell anaemia
>80%
88
What group of patients are more likely to have sickle cell anaemia
Patients with an Afro-Caribbean family background
89
Sickle cell crisis
Acute pain due to vaso-occlusion of the small vessels
90
What do people with sickle cell have an increased risk of
Infection
91
What is given long term management of sickle cell anaemia (4)
Folic acid supplements
Hydroxycarbamide
Regular transfusion
Prophylactic penicillin
92
How do we manage a sickle crisis (6)
Opiate analgesia
Hydration
Rest
Oxygen
Antibiotics if sign of infection
Red cell transfusion
93
Lung complication of sickle cell
Acute chest syndrome
94
What causes acute chest syndrome (3)
Infection
Fat embolism
Pulmonary infarction
95
Clinical presentation of acute chest syndrome
SOB, chest pain, pyrexia, hypoxia and consolidation on CXR
96
Haemolysis
Premature red cell destruction
97
What are the 5 general causes of haemolysis
Immune
Mechanical red cell destruction
Membrane defects
Abnormal red cell metabolism
Abnormal haemoglobin
98
Cold antibodies
IgM
99
Warm antibodies
IgG
100
Warm autoimmune causes of haemolysis
Idiopathic
SLE
lymphoproliferatice disorders
Drugs
infection
101
Name a drug that can cause haemolysis
Penicillin
102
Cold immune causes of haemolysis
Idiopathic
EBV
Lymphoproliferative disorders
103
State 2 alloimmune causes of haemolysis
Haemolytic transfusion reaction
Haemolytic disease of the newborn
104
Name some causes of mechanical red cell destruction
Disseminated intravascular coagulation
TTP
Leaking heart valve
Haemolytic uraemia syndrome
Burns
Infections
105
State membrane defect causes of haemolysis
Zieves syndrome
Vitamin E deficiency
Hereditary spherocytosis
106
What makes red cells more vulnerable to damage
Biconcave
Can’t generate new proteins as no nucleus
Limited metabolic reserve as no mitochondria
107
Haemolytic anaemia
Increased rate of red cell destruction exceeding bone marrow capacity for red cell production causes a fall in haemoglobin
108
State 3 consequences of haemolysis
- reticulocytosis by bone marrow
- bone marrow erythroid hyperplasia
- excess red cell break down products
109
What are the 2 main classifications of haemolysis
Intravascular and extravascular
110
What happens to red blood cells in extravascular haemolysis
Red cells are taken up by reticuloendothelial system
111
Are products normal or abnormal in extravascular haemolysis
Normal but generated in access
112
Are products of intravascular haemolysis normal or abnormal
Abnormal
113
Consequence of extravascular haemolysis
Hyperplasia at the site of destruction (splenohepatomegaly)
114
What happens to red cells in intravascular haemolysis
Red cells destroyed within the circulation spilling their contents
115
Clinical sign of haemoglobinurea
Pink urine that turns black on standing
116
Causes of intravascular haemolysis (4)
Incompatible blood transfusion
G6PD deficiency
Severe falciparum malaria
PNH, PCH
117
Reticulocyte count in haemolysis
Raised
118
Serum unconjugated bilirubin result in haemolysis
Raised
119
Serum hepatoglobins in haemolysis
Low
120
Urinary urobilinogen in haemolysis
Raised
121
What do spherocytes on blood film suggest
Membrane damage
122
What do red cell fragments suggest on blood film
Mechanical damage
123
What do Heinz bodies suggest on blood film
Oxidative stress due to G6PD deficiency
124
Coombs test result in haemolysis
Positive
