Blood disorders Flashcards

1
Q

What are the 2 major components of blood

A
  1. Formed elements (45%)

2. Plasma (55%)

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2
Q

What makes up the plasma in blood?

A
  1. Water
  2. Plamsa proteins
  3. Regulatory porteins
  4. Other sources
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3
Q

What makes up the formed elements of blood?

A
  1. Erythrocytes
  2. Leukocytes
  3. Platelets
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4
Q

What is another name for the former elements of blood?

A

Cellular component

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5
Q

What is hematocrit?

A

The proportion of whole blood that is made up of red blood cells

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6
Q

What percentage of the blood is made up of hematocrits?

A

42-47%

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7
Q

What is the difference between plasma and serum

A

Plasma includes fibrinogen

Serum is plasma without fibrinogen

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8
Q

What is the function of blood?

A
  1. Transportation of gases, waste, hormones and enzymes
  2. Fighting infection
  3. Homeostasis (Temperature, pH, volume)
  4. Haemostasis
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9
Q

How does the blood help with fighting infections?

A

It carries antibodies and leucocytes to site of infection

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10
Q

What is haemostasis?

A

Blood clotting

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11
Q

What is anaemia?

A

Haemoglobin concentration that is below a reference range for sex and age

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12
Q

Below what haemoglobin concentration would an adult be classified as anaemic?

A

Men: below 135g/L
Women: below 115g/L

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13
Q

What percentage of the world is anaemic?

A

32.9%

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14
Q

What can anaemia be caused by?

A
  1. Reduced red blood cell mass

2. Changes in plasma volumes

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15
Q

What can lead to changes in plasma volume?

A
  1. Dehydration

2. Pregnancy

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16
Q

What are some of the symptoms of anaemia?

A
  1. Fatigue, breathlessness
  2. Angina
  3. Intermittent claudication
  4. Palpations
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17
Q

What is intermittent claudication and what is it caused by?

A

Intermittent pain in the calf on walking due to reduced blood flow to the legs

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18
Q

When can anaemia be asymptomatic?

A

When it develops slowly

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19
Q

List some non specific signs of anaemia

A
  1. Pallor
  2. Tachycardia (Fast Heart rate)
  3. Cardiac murmur
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20
Q

What is the difference between a sign and symptom

A

A sign is a measurable abnormalities

A symptom is what the patient is feeling

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21
Q

List soem specific signs of anaemia

A
  1. Koilonychia
  2. Jaundice
  3. Leg ulcers
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22
Q

What is koilonychia?

A

Spooning of the fingernails

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23
Q

Is anaemia a diagnosis for pain?

A

No it is not a diagnosis in itself it is always an underlying cause

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24
Q

How can we investigate anaemia?

A

Look at the

  1. Peripheral blood
  2. Blood film
  3. Bone marrow
  4. Haematinics
  5. Iron status markers
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25
How do we use the peripheral blood to investigate anaemia
We can use: 1. Red cell indices (Hb, MVC) 2. White blood cell count 3. Platelet count 4. Reticulocyte count
26
What are haematincs
A nutrient required for haematopoesis ef Fe, B12, folate
27
Name the three nutrients haemantics usually refers to
1. Iron (Fe) 2. B12 3. Folate
28
How do we classify anaemia ?
1. By red cell size or volume (MCV) | 2. Underlying aetiology
29
What does MCV stand for
Mean corpuscular value
30
Name the different classifications of anaemia in regards to red cell size/ volume
1. Microcytic anaemia 2. Macrocytic anaemia 3. Normocyctic anaemia
31
What is microcytic anaemia ?
Anaemia where the red blood cells are small | MCV is below 80
32
What is macrocyclic anaemia?
Anaemia where the red blood cells are large | MCV is greater than 96
33
What are the units for MCV?
Femtoliters
34
What is normocytic anaemia?
Anaemia where the red blood cells are normal sized | MCV is between 80-96
35
Name the different classifications of anaemia in regards to the underlying aetiology
1. Haematinic deficiency | 2. Haemolysis
36
What is an Haematinic deficiency?
Impaired production of red blood cells due to a deficiency in: Fe B12 Folate
37
What is Haemolysis?
Increased LOSS of red blood cells
38
If a patient comes in with microytic anaemia what diagnosis might you reach?
1. Iron deficiency 2. Thalassaemia 3. Anaemia of chronic disease 4. Sideroblastic anaemia
39
If a patient comes in with macrocytic anaemia and large bone marrow what diagnosis might you reach?
Vitamin B12 or folate deficiency
40
If a patient comes in with macrocytic anaemia and normal bone marrow what diagnosis might you reach?
1. Alcohol reticulocytes 2. Liver disease 3. hypothyroidism 4. drug therapy
41
If a patient comes in with normocytic anaemia what diagnosis might you reach?
1. Acute blood loss 2. Anaemia of chronic disease 3. Chronic kidney disease 4. Autoimmune rheumatic disease 5. Marrow infiltration fibrosis 6. Endocrine disease 7. Haemolytic anaemia
42
Why is iron important in the body?
Iron is required for Hb synthesis
43
What is a normal cell sized mcv?
80-96
44
Name the most common type of anaemia
Iron deficiency anaemia
45
What causes iron deficiency anaemia
1. Blood loss 2. Increased demand eg pregnancy, growth 3. Decreased absorption (due to surgery) 4. Poor dietary intake
46
How can we investigate iron deficiency anaemia?
Look at: 1. Blood count 2. Blood film 3. Iron status
47
What do we expect to see in the blood count in a patient with iron deficiency anaemia
Microcytic hypochromic cells
48
What does hypo chromic mean when describing cells?
Pale cells
49
What do we expect to see in the blood film in a patient with iron deficiency anaemia
1. Poikilocytosis (variation in shape) | 2. Anisocytosis (variation in size)
50
Which proteins do we look for when looking at the iron status of a patient?
Ferritin and transferrin
51
Apart from iron deficiency anaemia what are the other causes of microcytic hypo chromic anaemia?
1. Anaemia chronic disease 2. Thalassaemia 3. Sideroblastic anaemia (these are rare)
52
Name the 2 terms we use to describe bone marrow
Megaloblastic | Normoblastic
53
What is another name for vitamin B12?
Cobalamin
54
What is the dietary requirement of vitamin b12?
2-3 micrograms a day
55
Give examples of food that contains vitamin b12
Meat Egg Milk
56
What does the body need to be able to absorb B12?
Intrinsic factor (IF)
57
What is intrinsic factor secreted by?
Gastric parietal cells
58
Where is vitamin b12 absorbed?
Small bowel
59
Anaemia where red blood cells are small is called what? What would the MCV be?
Microcytic anaemia | MCV below 80
60
Anaemia where red blood cells are large is called what? What would the MCV be?
Macrocyctic anaemia | MCV above 96
61
Anaemia where red blood cells are 'normal' is called what? What would the MCV be?
Normocyctic anaemia | MCV 80-96
62
How is vitamin b12 absorbed
1. B12 taken in from food 2. Binds to heptacorrin -> HC-Cbi 3. Pancreas enzymes release Cbi 4. Intrinsic factors from the gastric parietal cells binds to Cbi 5. the intrinsic factor-Cbi complex is then absorbed in the small bowel
63
What can cause vitamin B12 deficiency
1. Pernicious anaemia 2. Dietary- veganism 3. Surgery 4. Ileal disease 5. Parasites
64
What causes pernicious anaemia?
Loss of cheif/ parietal cells | This decreases the concentration of intrinsic factor ultimately reducing B12 absorption
65
Name the most common cause for vitamin B12 deficiency
Pernicious anaemia
66
How can we investigate vitamin B12 deficiency
Look at: 1. Blood count 2. Blood film 3. Bone marrow
67
What would you expect to see on a blood count of a patient with vitamin B12 deficiency?
Macrocytic cells (MCV greater than 96)
68
What would you expect to see on a blood film of a patient with vitamin B12 deficiency?
Oval macrocytes Hyper-segmented polymorphs Severe leukopenia (white blood cell loss)
69
What would you expect the bone marrow to look like if a patient with vitamin B12 deficiency?
Increased megaloblasts | Immature red cell precursor cells
70
How do we treat B12 deficiency
B12 injection
71
Why does b12 deficiency cause macrocytic anaemia
As B12 needed for DNA synthesis without it you can get abnormal nuclear maturation/ cell division Cells end up making more protein than DNA so balloon up and get big
72
Which vitamins are needed for DNA synthesis?
Vitamin b12 | Folate
73
In which food is folate found?
Fruits and vegetables
74
Why is folate important
Required for DNA synthesis
75
Give some causes of folate deficiency?
1. Malnutrition 2. Malabsorption (Crohn's disease) 3. Increased demand (pregnancy, breastfeeding, cancer) 4. Some drugs (some anti-epileptics)
76
What is haemolytic anaemia?
When the lifespan of the red blood cell is shortened | It can be hereditary of acquired
77
Name some causes of Hereditary haemolytic anaemia
1. Haemoglobin synthesis abnormalities 2. Red cell membrane defects 3. Metabolic defects
78
Give an example of a haemoglobin synthesis abnormality
Sickle cel anaemia
79
Give an example of a red cell membrane defects
Hereditary spherocytosis/ elliptocytosis
80
Give an example of a metabolic defects
Glucose-6-phosphate dehydrogenase deficiency
81
What causes sickle cell anaemia?
HbS single gene mutation
82
Which gene is mutated in sickle cell anaemia
Valine for glutamic acid substituted 6th cocoon of beta globing chain
83
What can make the effects of sickle cell anaemia worse?
``` Infection Dehydration Cold Acidosis Hypoxia ```
84
What can sickle cell anaemia result in?
1. Decreased Red blood cell survival | 2. Obstruction of small vessels and tissue infarction
85
What are the clinical features of sickle cell anaemia?
1. Anaemia 2. Vaso-occlusive crises 3. Acute chest syndrome (30% of patients) 4. Long term complications
86
Give examples of vaso-occlusive crises that are features of sickle cell anaemia
1. Acute pain in the hands and feet (dactylitis) 2. Severe pain femur, humerus, vertebrae, ribs and pelvis 3. Hospital admissions due to pain
87
What is acute chest syndrome?
Infection, fat embolism from necrotic marrow or pulmonary infarction Can lead to shortness of breath, chest pain and hypoxia
88
What would you expect to see on a blood count for a patient with sickle cell anaemia?
Normocytic anaemia
89
What would you expect to see on a blood film for a patient with sickle cell anaemia?
Sickled red cells
90
How do you treat sickle cell anaemia?
We can't treat it but we manage the symptoms depending on the complications 1. Hospitalisation 2. Supportive IV fluids 3. Analgesia
91
What are some of the oral manifestations of anaemia
1. Angular chelitis 2. Atrophic glossitis 3. Recurrent aphthous stomatitis (RAS)
92
What is angular chelitis
Soreness at corners of the mouth
93
What is Atrophic glossitis
Smooth tongue
94
What is Recurrent aphthous stomatitis (RAS)
Ulcers in the mouth that 10-14 days after healing
95
What are Recurrent aphthous stomatitis (RAS) associated with?
Iron, B12 and folate deficiencies
96
What is haemostasis?
Process by which blood clots at the sites of vascular injury
97
Why is haemostasis important?
As circulating blood volume is finite
98
Name the stages of haemostasis
1. Vasoconstriction 2. Primary haemostasis (Platelet plug formation) 3. Secondary haemostaisis (fibrin meshwork) 4. Clot stabilisation / resorption
99
What is vasoconstriction and why is it important?
It is construction of the blood vessels | It is important as it reduces blood flow
100
What is released during vasoconstriction?
Neurogenic/ factor secretion eg endothelin
101
What is primary haemostasis?
Formation of platelet plus
102
How is the platelet plug formed?
1. Endothelial is damaged due to injury leading to exposure of collagen 2. Von Willebrand factor binding occurs 3. This induces platelets to adhere and activate changing in to a spiky shape 4, Platelets secrete granules attracting more platelets 5. Aggregate to form a platelet plug
103
What is secondary haemostasis?
Deposition of fibrin meshwork
104
What happens in secondary haemostasis
1. Tissue factor is exposed 2. Activates coagulation cascade 3. Trhombin cleaves to fibrinogen converting it into fibrin which forms a meshwork 4. This activates more platelets 5 Red blood cells become trapped
105
What is the importance of fibrin?
stabilises platelets and activates more platelets
106
Defects in blood clotting can result from...?
1. Vessel wall abnormalities 2. Platelet deficiency 3. Abnormal coagulation 4. Combination of the above
107
How can the walls of the blood vessels get damaged?
1. Infection 2. Drug reactions 3. Scurvy 4. Hereditary haemorrhagic telengectasia 5. Pericascular amyloidosis
108
Name a disease can cause microbial damage in the blood vessels
Meningcoccal septicaemia
109
What is platelet deficiency called?
Thombocytopaenia
110
Define platelet deficiency
When a persons platelet concentration is below 150x10^9 per L
111
What causes platelet deficiency ?
1. Decreased production 2. Decreased survival 3. Sequestration 4. Dilution (due to blood transfusions)
112
When are less platelets produced by the body?
1. Aplastic anaemia 2. Leukaemia 3. Drugs 4. Alcohol
113
What us the significance of factor VIII?
It is a cofactor for the coagulation cascasde | It converts fibrolgin into fibrin
114
What is the significance of the Von Willebrand factor (vWF)
It is important as it plays a role in platelet binding
115
Name the 2 substances that are essential for blood clotting
Factor VIII | Von Willebrand factor (vWF)
116
When can abnormal coagulation occur
1. Hereditary disorders 2. Acquired deficiencies 3. Liver disease
117
Give examples of hereditary disorders that can lead to abnormal coagulation
1. Von Willebrand disease | 2. Haemophillia A and B (
118
What does haemophilia A and B affect?
Factor VIII and IX
119
Name the most common inherited bleeding disorder
Von Willebrand disease
120
How is Von Willebrand disease passed on?
Autosomal dominent
121
What is Thombocytopaenia?
Platelet deficiency
122
How many types of Von Willebrand disease are there and which is most common
3 types Type 1 is the most common Type 3 is the most severe
123
How are the platelets affected in Von Willebrand disease?
Their function is impaired
124
How is Von Willebrand disease treated?
1. Desmopressin (promotes Von Willebrand factor production) | 2. Plasma concentration infusions
125
How is haemophilia A passed on and what does it affect?
X linked recessive | Causes factor VIII mutations
126
What can a person with haemophilia A suffer from?
1. Easy bruising 2. Haemorrhage post trauma. surgery 3. Spontaneous Haemorrhage
127
How do we treat haemophilia A?
Infusion of factor VIII
128
What is another name for haemophilia B?
Christmas disease
129
How is haemophilia B passed on and what does it affect?
X linked recessive | Causes factor IX deficiency
130
How is haemophilia B treated
Recombinant factor IX
131
what is disseminated intravascular coagulation?
A Thrombohaemorrhagic disorder
132
What is a Thrombohaemorrhagic disorder?
A disorder that results in excessive and widespread activation of coagulation leading to the formation of a thrombi in microvasculature
133
Is disseminated intravascular coagulation a diagnosis?
NO you need to find and treat the underlying cause of this