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Red Cells Flashcards

1
Q

What substances are required for red cell production?

A

Iron
B12
Folic acid
Erythropoietin

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2
Q

Where does red cell breakdown occur?

A

Spleen

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3
Q

What system is involved in red cell breakdown?

A

Reticuloendothelial system

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4
Q

Red cell life span

A

120 days

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5
Q

What is bilirubin bound to in the plasma?

A

Albumin

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6
Q

What is haem?

A

The part of haemoglobin containing iron

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7
Q

What is contained in the mature red blood cell?

A

Membrane
Enzymes
Haemoglobin

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8
Q

Where are genetic defects described in the RBC?

A

Red cell membrane
Metabolic pathways (enzymes)
Haemoglobin

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9
Q

Which proteins are responsible for maintaining red cell shape and deformability?

A

Skeletal proteins

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10
Q

What can defects in skeletal proteins can lead to?

A

Increased cell destruction

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11
Q

What is hereditary spherocytosis?

A

Red cells are spherical
Removed from circulation by the RE system (extravascular)
Most commonly autosomal dominant
Defects in 5 different structural proteins

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12
Q

Presentation of hereditary spherocytosis

A

Anaemia
Jaundice (neonatal)
Splenomegaly
Pigment gallstones

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13
Q

Treatment of hereditary spherocytosis

A

Folic acid (increased requirements)
Transfusion
Splenectomy if anaemia very severe

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14
Q

Membrane disorders

A

Hereditary spherocytosis
Hereditary Elliptocytosis (rather than spheres)
Hereditary Pyropoikilocytosis (all different sizes)
South East Asian Ovalocytosis

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15
Q

What is the function of glycolysis?

A

Provides energy

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16
Q

What are the enzyme pathways used by red cells?

A

Glycolysis

Pentose Phosphate shunt

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17
Q

What is the function of the pentose phosphate shunt (/Glucose 6 Phosphate Dehydrogenase (G6PD))?

A

Protects cell proteins (haemoglobin) from oxidative damage

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18
Q

Why is G6PD deficiency common?

A

Confers protection against malaria

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19
Q

What is G6PD deficiency?

A

Commonest disease causing enzymopathy in the world
Cells vulnerable to oxidative damage
X Linked

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20
Q

Presentation of G6PD deficiency

A

Variable degrees of anaemia
Neonatal Jaundice
Splenomegaly
Pigment Gallstones

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21
Q

What is pyruvate kinase deficiency?

A

Reduced ATP - glycolytic pathway
Increased 2,3-DPG
Cells rigid

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22
Q

When does haemoglobin give up oxygen to tissues?

A

Decreased pH
Increased temperature
Increased CO2

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23
Q

What are the normal proportions of adult Hb?

A 
Adult haemoglobin (HbA) composed of haem molecule and:
Hb A  (αααα)  – 97% - 4 alpha chains
Hb A2 (ααδδ) – 2% - 2 alpha, 2 delta
Hb F  (ααγγ)  – 1% (foetal Hb) - 2 alpha, 2 gamma
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24
Q

Haemoglobinopathies

A

Inherited abnormalities of haemoglobin synthesis
Reduced or absent globin chain production (thalassaemia)
Mutations leading to structurally abnormal globin chain (sickle cell disease)

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25
What is the sickle cell disease composition?
Sickle haemoglobin (HbS) composed of haem molecule and 2 α chains 2 β (sickle) chains
26
Consequences of HbS Polymerisation
Red cell injury, cation loss, dehydration => haemolysis => Endothelial activation Promotion of inflammation Coagulation activation Dysregulation of vasomotor tone by vasodilator mediators (NO) => vaso-occlusion
27
Sickle cell presentation
``` Painful Vaso-occlusive crises - Bone Chest Crisis Stroke Increased infection risk - Hyposplenism Chronic haemolytic anaemia - Gallstones - Aplastic crisis Sequestration crises - Spleen - Liver ```
28
Management of sickle cell disease
``` Life long prophylaxis - Vaccination - Penicillin (and malarial) prophylaxis - Folic acid Acute Events - Hydration - Oxygenation - Prompt treatment of infection - Analgaesia (opiates, NSAIDs) - Blood transfusion ```
29
Disease modifying drugs in sickle cell disease
Hydroxycarbamide
30
What is thalassaemia?
Reduced or absent globin chain production
31
Homozygous alpha zero thalassaemia (α0/α0 )
No alpha chains | Hydrops Fetalis – incompatible with life
32
Beta thalassaemia major (Homozygous beta thalassaemia)
No beta chains | Transfusion dependent anaemia
33
Thalassaemia minor (common)
“Trait” or carrier state | Hypochromic microcytic red cell indices
34
Beta thalassaemia major
Severe anaemia - Present at 3-6 months of age - Expansion of ineffective bone marrow - Bony deformities - Splenomegaly - Growth retardation Life expectancy untreated or with irregular transfusions <10 years
35
Beta thalassaemia major treatment
4-6 weekly blood transfusions | Causes iron overload so chelation therapy required
36
Chelation agents
Removes excess iron DFO DFP DFX
37
Sideroblastic anaemia
Defects in mitochondrial steps of haem synthesis ALA synthase mutations Hereditary Aquired – (most common) a form of myelodysplasia
38
General clinical features of anaemia due to reduced oxygen delivery to tissues
``` Tiredness/pallor Breathlessness Swelling of ankles Dizziness Chest pain ```
39
What are the overall causes of anaemia?
Bone marrow Red cell Destruction/loss
40
Causes of anaemia involving the bone marrow
Cellularity Stroma Nutrients
41
Causes of anaemia involving the red blood cells
Membrane Haemoglobin Enzymes
42
Causes of anaemia involving destruction/loss
Blood loss Haemolysis Hypersplenism
43
What are red cell indices
Automated measurement of red cell size and haemoglobin content
44
MCH
Mean cell haemoglobin
45
MCV
Mean cell volume (cell size)
46
Morphological descriptions of anaemia
Hypochromic, microcytic Normochromic, normocytic Macrocytic
47
Blood film required for hypochromic, microcytic
Serum ferritin
48
Blood film required for normochromic, normocytic
Reticulocyte count
49
Blood film required for macrocytic anaemia
B12/folate | Bone marrow
50
Diagnosis for low serum ferritin in hypochromic microcytic anaemia
Iron deficiency
51
Diagnosis for normal or increased serum ferritin in hypochromic microcytic anaemia
Thalassaemia secondary anaemia
52
What happens to absorbed iron in the body?
Bound to mucosal ferritin or Transported across the basement membrane by ferroportin Then bound to transferrin in plasma Stored as Ferritin - mainly in liver
53
What is hepcidin
Reduces intestinal iron absorption and mobilisation from reticuloendothelial cells Synthesised in hepatocytes in response to ↑iron levels and inflammation
54
What is the commonest cause of anaemia?
Iron deficiency
55
Clinical features of iron deficiency
Koilonychia Atrophic tongue Angular cheilitis
56
Causes of iron deficiency
``` GI blood loss Menorrhagia Malabsorption - gastrectomy - coeliac disease ```
57
Diagnosis for normal/low reticulocyte count in normochromic normocytic anaemia
Secondary anaemia Hypoplasia Marrow infiltration
58
Diagnosis for increased reticulocyte count in normochromic normocytic anaemia
Acute blood loss | Haemolysis
59
What is secondary anaemia?
"Anaemia of chronic disease" | Mainly normochromic normocytic, also hypochromic microcytic
60
Why is there a decreased haemoglobin in haemolytic anaemia?
Accelerated cell destruction
61
Why is there an increase in reticulocyte count in haemolytic anaemia?
Compensation by bone marrow as a result of the decreased haemoglobin
62
What is the reticulocyte count?
measures how fast reticulocytes are made by the bone marrow and released into the blood
63
What are reticulocytes?
Immature red blood cells
64
Congenital causes of haemolytic anaemia
``` Hereditary spherocytosis (HS) Enzyme deficiency (G6PD deficiency) Haemoglobinopathy (HbSS) ```
65
Extravascular acquired causes of haemolytic anaemia
Auto-immune haemolytic anaemia
66
Intravascular acquired causes of haemolytic anaemia
Mechanical eg.artificial valve Severe infection/DIC PET/HUS/TTP
67
TTP
Thrombotic thrombocytopenia purpura
68
What is the purpose of the direct antiglobulin test?
Detects antibody or complement on red cell membrane | Implies immune basis for haemolysis
69
What does the reagent in a direct antiglobulin test contain?
Anti-human IgG | Anti-complement
70
Haemolytic anaemia management
Support marrow function - Folic acid Correct cause - Immunosuppression if autoimmune - Remove site of red cell destruction (splenectomy) - Treat sepsis, leaky prosthetic valve, malignancy etc. if intravascular Consider transfusion
71
What are the possible results of a B12/folate assay?
Megaloblastic | Non-megaloblastic
72
Megaloblastic macrocytic anaemia causes
B12 deficiency | Folate deficiency
73
Non-megaloblastic macrocytic anaemia causes
Myelodysplasia Marrow infiltration Drugs
74
What does dietary B12 bind to?
Intrinsic factor
75
Which cells secrete intrinsic factor?
Gastric parietal cells
76
What binds to B12, where and why?
Transcobalamin II In portal circulation For transport to marrow and other tissues
77
Presentation of megaloblastic anaemia
"Lemon yellow" tinge Bilirubin, LDH Red cells friable
78
What is pernicious anaemia?
Autoimmune disease Antibodies against intrinsic factor (diagnostic) or gastric parietal cells (less specific) Malabsorption of dietary B12
79
Megaloblastic anaemia treatment
Replace vitamin B12 deficiency - B12 IM injection Folate deficiency - oral folate supplement (ensure B12 normal if neuropathic symptoms)
80
Other causes of macrocytosis
``` Alcohol Drugs (Methotrexate, Antiretrovirals, hydroxycarbamide) Disordered liver function Hypothyroidism Myelodysplasia ```
81
What is aplastic anaemia?
Autoimmune disease in which the body fails to produce blood cells in sufficient numbers Deficiency of all blood cell types
82
What is aplastic crisis?
The body does not make enough new red blood cells to replace the ones that are already in the blood
83
What is the commonest cause of a macrocytosis?
Liver disease

Haematology (18 decks)

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