PATHOLOGY- Blood disorders Flashcards

(123 cards)

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 proteins
  4. Other sources
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3
Q

What makes up the formed elements of blood?

A
  1. Erythrocytes (99%)
  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 is the equation to work out the haematocrit

A

% RBC / ratio of RBC volume to total BV

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7
Q

what is another word for haematocrit

A

packed cell volume (PCV)

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8
Q

What percentage of the blood is made up of hematocrits?

A

42-47%

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9
Q

What is the difference between plasma and serum

A

Plasma includes fibrinogen
Serum is plasma without fibrinogen (e.g. after blood clot)

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10
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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11
Q

How does the blood help with fighting infections?

A

It carries antibodies and leukocytes to site of infection

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12
Q

What is haemostasis?

A

Blood clotting

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13
Q

What is anaemia?

A

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

usually a loss in RBC mass

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14
Q

when are there apparent [Hb] changes

A

when there are changes in plasma volume
plasma vol dec=apparent inc[Hb]
plasma vol inc=apparent dec[Hb]

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15
Q

how do you classify anaemia

A

By red cell size / volume (MCV)

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16
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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17
Q

What can anaemia be caused by?

A
  1. Reduced red blood cell mass
  2. Changes in plasma volumes
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18
Q

What can lead to changes in plasma volume?

A
  1. Dehydration
  2. Pregnancy
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19
Q

What are some of the symptoms of anaemia?

A
  1. Fatigue, breathlessness
  2. Angina
  3. Intermittent claudication
  4. Palpations
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20
Q

When can anaemia be asymptomatic?

A

When it develops slowly

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21
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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22
Q

How do we use the peripheral blood to investigate anaemia

A

We can use:

  1. Red cell indices (Hb, MVC)
  2. White blood cell count
  3. Platelet count
  4. Reticulocyte count
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23
Q

What are haematincs

A

A nutrient required for haematopoesis e.g. Fe, B12, folate

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24
Q

Name the three nutrients haemantics usually refers to

A
  1. Iron (Fe)
  2. B12
  3. Folate
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25
How do we classify anaemia ?
1. By red cell size or volume (MCV) 2. Underlying aetiology
26
What does MCV stand for
Mean corpuscular value
27
Name the different classifications of anaemia in regards to red cell size/ volume
1. Microcytic anaemia 2. Macrocytic anaemia 3. Normocyctic anaemia
28
What is microcytic anaemia ?
Anaemia where the red blood cells are small MCV is below 80
29
What is macrocyclic anaemia?
Anaemia where the red blood cells are large MCV is greater than 96
30
What are the units for MCV?
Femtoliters
31
What is normocytic anaemia?
Anaemia where the red blood cells are normal sized MCV is between 80-96
32
Name the different classifications of anaemia in regards to the underlying aetiology
Impaired production of red cells - Haematinic deficiency (e.g. Fe, B12, folate) - most common Increased loss of red cells - e.g. haemolysis
33
What is an Haematinic deficiency?
Impaired production of red blood cells due to a deficiency in: Fe B12 Folate | most common
34
What is Haemolysis?
when blood breaks down abnormally
35
What happens if the Fe store balance in the body is disturbed
- impairs the absorption capacity - increases the excretion - makes you vulnerable to Fe deficiency
36
what is hypochromic
small pale cells MCH <27 | MCH= Mean corpusucalr haemaglobin
37
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
38
If a patient comes in with macrocytic anaemia and large bone marrow what diagnosis might you reach?
Vitamin B12 or folate deficiency
39
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
40
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
41
Why is iron important in the body?
Iron is required for Hb synthesis
42
What is a normal cell sized mcv?
80-96
43
Name the most common type of anaemia
Iron deficiency anaemia
44
What causes iron deficiency anaemia
1. Blood loss 2. Increased demand eg pregnancy, growth 3. Decreased absorption (due to surgery) 4. Poor dietary intake
45
How can we investigate iron deficiency anaemia?
Look at: 1. Blood count 2. Blood film 3. Iron status
46
What do we expect to see in the blood count in a patient with iron deficiency anaemia
Microcytic hypochromic cells
47
What does hypo chromic mean when describing cells?
Pale cells
48
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)
49
Which proteins do we look for when looking at the iron status of a patient?
Ferritin and transferrin
50
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)
51
Name the 2 terms we use to describe bone marrow
Megaloblastic Normoblastic
52
What is another name for vitamin B12?
Cobalamin
53
What is the dietary requirement of vitamin b12?
2-3 micrograms a day
54
Give examples of food that contains vitamin b12
Meat Egg Milk
55
What does the body need to be able to absorb B12?
Intrinsic factor (IF)
56
What is intrinsic factor secreted by?
Gastric parietal cells
57
Where is vitamin b12 absorbed?
Small bowel
58
Anaemia where red blood cells are small is called what? What would the MCV be?
Microcytic anaemia | MCV below 80
59
Anaemia where red blood cells are large is called what? What would the MCV be?
Macrocyctic anaemia | MCV above 96
60
Anaemia where red blood cells are 'normal' is called what? What would the MCV be?
Normocyctic anaemia | MCV 80-96
61
How is vitamin b12 absorbed
1. B12 taken in from food 2. Binds to heptacorrin -> HC-Cbl 3. Pancreas enzymes release Cbl 4. Intrinsic factors from the gastric parietal cells binds to Cbl 5. the intrinsic factor-Cbl complex is then absorbed in the small bowel
62
What can cause vitamin B12 deficiency
1. Pernicious anaemia 2. Dietary- veganism 3. Surgery 4. Ileal disease 5. Parasites 6. Dtugs e.g. PPIs
63
What causes pernicious anaemia?
Loss of cheif/ parietal cells This decreases the concentration of intrinsic factor ultimately reducing B12 absorption
64
Name the most common cause for vitamin B12 deficiency
Pernicious anaemia
65
How can we investigate vitamin B12 deficiency
Look at: 1. Blood count 2. Blood film 3. Bone marrow
66
What would you expect to see on a blood count of a patient with vitamin B12 deficiency?
Macrocytic cells (MCV greater than 96)
67
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)
68
What would you expect the bone marrow to look like if a patient with vitamin B12 deficiency?
Increased megaloblasts Immature red cell precursor cells
69
How do we treat B12 deficiency
B12 injection
70
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
71
Which vitamins are needed for DNA synthesis?
Vitamin b12 | Folate
72
In which food is folate found?
Fruits and vegetables
73
Why is folate important
Required for DNA synthesis
74
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)
75
what is the most common cause of normocytic anaemia
haemolytic anaemia
76
What is haemolytic anaemia?
When the lifespan of the red blood cell is shortened Abonrmal breakdown of red blood cell | It can be hereditary or acquired
77
Name some causes of Hereditary haemolytic anaemia
1. Haemoglobin synthesis abnormalities e.g. sickle cell 2. Red blood cell membrane defects 3. Metabolic pathway defects
78
Give an example of a haemoglobin synthesis abnormality
Sickle cell anaemia
79
What causes sickle cell anaemia?
HbS single gene mutation
80
Which gene is mutated in sickle cell anaemia
Valine for glutamic acid substituted 6th codon of beta-globin chain
81
what causes the sickle shaped cells in sickle cell anaemia
1. Abnormal form of Hb present -> HbS 2. When there is low conc of O2, HbS molecules polymerise (stick together), deforming the red blood cells 3. RBCs become sickle shaped cells
82
What can make the effects of sickle cell anaemia cause a vaso occlusive crises (VOC)?
Infection Dehydration Cold Acidosis Hypoxia
83
What can sickle cell anaemia result in?
1. Decreased Red blood cell survival (haemolysis) 2. Obstruction of small vessels and tissue infarction
84
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
85
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
86
What is acute chest syndrome?
Infection, fat embolism from necrotic marrow or pulmonary infarction Can lead to shortness of breath, chest pain and hypoxia
87
What would you expect to see on a blood count for a patient with sickle cell anaemia?
Normocytic normochromic anaemia
88
What would you expect to see on a blood film for a patient with sickle cell anaemia?
Sickled red cells
89
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
90
What are some of the oral manifestations of anaemia
1. Angular chelitis 2. Atrophic glossitis 3. Recurrent aphthous stomatitis (RAS)
91
What is angular chelitis
Soreness at corners of the mouth
92
What is Atrophic glossitis
Smooth tongue
93
What is Recurrent aphthous stomatitis (RAS)
Ulcers in the mouth that reoccur 10-14 days after healing
94
What are Recurrent aphthous stomatitis (RAS) associated with?
Iron, B12 and folate deficiencies
95
What is haemostasis?
Process by which blood clots at the sites of vascular injury
96
Why is haemostasis important?
As circulating blood volume is finite
97
Name the stages of haemostasis
1. Vasoconstriction 2. Primary haemostasis (Platelet plug formation) 3. Secondary haemostaisis (fibrin meshwork) 4. Clot stabilisation / resorption
98
What is vasoconstriction and why is it important?
It is constriction of the blood vessels It is important as it reduces blood flow
99
What is secreted during vasoconstriction?
Neurogenic/ factor secretion eg endothelin
100
What is primary haemostasis?
Formation of platelet plus
101
How is the platelet plug formed in primary haemostasis
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
102
What is secondary haemostasis?
Deposition of fibrin meshwork
103
What happens in secondary haemostasis
1. Tissue factor is exposed 2. Activates coagulation cascade 3. Thrombin cleaves to fibrinogen converting it into fibrin which forms a meshwork 4. This activates more platelets 5. Red blood cells become trapped
104
What is the importance of fibrin?
stabilises platelets and activates more platelets
105
what is the coagulation cascade stimulated by
exposed tissue factor
106
what does the coagulation cascade produce
1. prothrombin converted in to thrombin 2. thrombin then acts to convert fibrinogen into a fibrin clot (fibrin glues the platelets together)
107
4 things that can cause defects in blood clotting
* Vessel wall abnormalities * Platelet deficiency / dysfunction * Abnormal coagulation * Combinations of above
108
What us the significance of factor VIII?
It is a cofactor for the coagulation cascasde It converts fibrolgin into fibrin
109
What is the significance of the Von Willebrand factor (vWF)
binds platelets but also factor VIII
110
Name the 2 substances that are essential for blood clotting
Factor VIII Von Willebrand factor (vWF)
111
Give examples of hereditary disorders that can lead to abnormal coagulation
1. Von Willebrand disease 2. Haemophillia A
112
Name the most common inherited bleeding disorder
Von Willebrand disease
113
How is Von Willebrand disease passed on?
Autosomal dominent
114
what are the 3 types of vW disease
* T1 (80%) - JWF * T2 (20%) = dysfunction * T3 (rare/severe) = no WF
115
What is Thombocytopaenia?
Platelet deficiency
116
How are the platelets affected in Von Willebrand disease?
Normal platelet count but defective function
117
How is Von Willebrand disease treated?
1. Desmopressin (promotes Von Willebrand factor production) 2. Plasma concentration infusions
118
How is haemophilia A passed on and what does it affect?
X linked recessive Causes factor VIII mutations leading to impaired coagulation
119
What can a person with haemophilia A suffer from?
1. Easy bruising 2. Haemorrhage post trauma. surgery 3. Spontaneous Haemorrhage
120
How do we treat haemophilia A?
Infusion of factor VIII
121
what is disseminated intravascular coagulation?
A Thrombohaemorrhagic disorder
122
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
123
Is disseminated intravascular coagulation a diagnosis?
NO you need to find and treat the underlying cause of this