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Y3- I & W > pathology: blood disorders > Flashcards

pathology: blood disorders Flashcards

1
Q

what is blood

A

make up from
55% plasma
45% formed elements

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2
Q

what can we find in plasma

A

water
plasma proteins
regulatory proteins
other solutes

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3
Q

what can we find in the formed elements

A

erythrocytes
leukocytes
platelets

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4
Q

what is the difference between serum and plasma

A

serum lacks fibrinogen

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5
Q

what is the point of blood

A

transportation
infection
homeostasis
haemostasis

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6
Q

what is anaemia

A

is a haemoglobin concentration that is below the reference range for the appropriate sex and age

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7
Q

what is the normal haemoglobin conc for men

A

<135g/L

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8
Q

what is the normal haemoglobin conc for women

A

<115g/L

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9
Q

what is anaemia caused by

A

a decrease in RBC mass

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10
Q

what are some other things that cause anaemia

A

changes in plasma volume from:
dehydration
pregnancy
saline usage

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11
Q

what happens when someone is dehydrated

A

the plasma volume reduces–> looks like high haemoglobin levels

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12
Q

what happens when someone is pregnant

A

high plasma volume therefore looks like low haemoglobin levels

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13
Q

what are the symptoms of anaemia

A 
○ Fatigue 
	○ Breathlessness
	○ Lightheaded
	○ Angina 
	○ Intermittent claudication 
	○ Palpitations 
May be asymptomatic if developed slowly
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14
Q

what is intermittent claudication

A

a pt with peripheral vascular disease gets intermittent pain in the lower leg or calf when walking, relieved by rest

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15
Q

what are non specific signs of anaemia

A

pain
tachycardia
cardia murmur

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16
Q

what are specific signs of anaemia

A

koilonychia- specific for iron deficient anaemia
jaundice- can be specific for haemolytic anaemia
leg ulcers- specific for sickle cell anaemia

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17
Q

is anaemia a diagnosis

A

NO it is a manifestation of an underlying disease

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18
Q

what investigations can we do for anaemia

A 
we can examine the peripheral blood 
blood film 
bone marrow 
additional tests such as- haematinics 
iron status markers
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19
Q

what can we see in the peripheral blood for anaemia

A

Such as the red cell indices e.g mean corpuscular volume, Hb
WCC
Platelet count
Reticulocyte count

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20
Q

what can we see on a blood film

A

allows us determine the size and shape

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21
Q

what can we see in the bone marrow

A

abnormal maturations of the RBCs

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22
Q

what is haematinics

A

any nutrient that is required for haematopoesis

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23
Q

what nutrients are needed for haematopoesis

A

iron
b12
folate

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24
Q

how do we classify anaemia

A

by red cell size/volume

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25
if the cells are small what do we call the anaemia
microcytic anaemia if they are smaller than 80 femotolitres
26
if the cells are large what do we call the anaemia
macrocytic anaemia if they are larger than 96 femtolitres
27
what do we call the anaemia if it is between 80-96 femtolitres
then you have normocytic anaemia
28
describe iron deficient anaemia
associated with due to microcytic anaemia
29
what are the cuases of iron deficient anaemia
blood loss increased demand eg pregnancy decreased absroption eg surgery poor dietary intake
30
how do we investigate iron deficient anaemia
blood count blood film iron status
31
what do we expect to see on the blood count of someone with iron deficient anaemia
microcytic anaemia | hypochromic anaemia
32
what do we expect to see on the blood film of someone with iron deficient anaemia
poikilocytosis- change in shape | anisocytosis- variation in size
33
what are other causes of iron deficient anaemia
anaemia chronic disease thalassaemia sideroblastic anaemia
34
Describe megaloblastic anaemia
associated with macrocytic anaemia | associated with vitamin B12
35
how much vitamin b12 do we need a day
2-3micrograms/day
36
how much vit b12 can we take in a day
20 micrograms if we eat meat/eggs and it is stored in the liver
37
how do we absorb vitamin b12
by IF in the small bowel | binds to IFb12 complex absorbed in the small bowel
38
what is IF secreted by
gastric parietal cells
39
the process of vitamin b12 absorption
1. b12 comes from food 2. binds to haptocorrin- HC-CBL 3. pancreatic enzymes release Cbl 4. IF secreted from gastric parietal cells 5. IF-cbl complex absorbed in the small bowel
40
what is pernicious anaemia
autoimmune condition due to the loss of parietal cells in the stomach less IF produced therefore less B12 absorption
41
what do we see in the blood count of pernicious anaemia
large MCV macrocytic anaemia
42
what do we see in the blood film of pernicious anaemia
oval macrocytes hyper segmented polymorphs severe deficiency- leukopenia/thrombocytopenia
43
what do we see in the bone marrow of pernicious anaemia
hypersegmented polymorphs | increased megaloblasts
44
what is the treatment of pernicious anaemia
IM b12 injections
45
why does b12 deficiency/ folate deficiency cause macrocytic anaemia
It causes macrocytic anaemia as there is an XS of proteins made in the cells and impaired nuclear maturation which causes the cell to become big.
46
what are the causes of folate deficiency
malnutrition malabsorption increased demand some drugs- anti-epileptics
47
give an example of normocytic anaemia
haemolytic anaemia
48
describe haemolytic anaemia
the RBCS have a short life span | it is an acquired disease
49
what ate the different types of haemolytic anaemia
haemoglobin synthesis abnormalities red cell membrane defects metabolic defects
50
give an example of anaemia that occurs from haemoglobin synthesis abnormalities
sickle cell anaemia
51
give an example of anaemia that occurs from red cell membrane defects
hereditary spherocytosis
52
give an example of anaemia that occurs from metabolic defects
Glucose-6 phosphate dehydrogenase deficiency
53
describe sickle cell anaemia
involves a single gene substitution mutation | where valine is subed for glutamic acid on the 6th codon of the beta globin chain
54
what happens when there is low levels of o2 in people with sickle cell anaemia
the HbS molecule sticks together
55
how can the sickle shape occur
``` Infection Dehydration Acidosis Hypoxia Cold ```
56
what does sickle cell anaemia result in
haemolysis | obstruction of the small vessels
57
clinical features of sickle cell anaemia
· Anaemia · Vaso occlusive crises: · Which includes pain in the hands and feet · Sever pain femur, humerus, vertebrae, ribs and pelvis resulting in hosp admin · Acute chest syndrome: Infection or a fat embolism from necrotic marrow
58
what are the symptoms of sickle cell anaemia
shortness of breath chest pain hypoxia
59
investigaations of sickle cell anaemia
blood count- normocytic anaemia | blood film: sickled red cells
60
treatment of sickle cell anaemia
depends on the complications
61
oral manifestations of anaemia
angular chelitis atrophic glossitis recurrent apthous stomatitis
62
what is angular chelitis
soreness of the corners of the mouth
63
what is atrophic glossitis
smooth tongue
64
what is recurrent apthous stomatitis
ulcers in the mouth for 10-14 days seen with b12/folate, iron deficiencies check the haematinics
65
what is haemostasis
blood clotting at a site of injury
66
what is the sequence of blood clotting
1. vasoconstriction 2. platlet plug formation 3. fibrin meshwork 4. insoluble firinogenin meshwork
67
what happens in vasoconstriction
Reduces the blood flow Endothelin is released which leads to constriction of the blood vessels It is a temporary fix
68
what happens in platelet plug formation
There is endothelial disruption and the collagen is exposed VWF biding The platelets adhere and change shape More granules are released and more platelets are released Aggregate to form a plug
69
what happens in fibrin meshwork
The Tissue factor is exposed Activates the coagulation cascade Thrombin--> cleaves fibrinogen--> insoluble fibrin and then meshwork RBC entrapment
70
how can bleeding disorders occur
``` from defects in the vessel wall abnormalities platelet deficiency abnormal coagulation acquired deficiencies liver disease ```
71
describe vessel wall abnormalities
• Can be from infection eg meningococcal septicaemia • Drug reactions( Henoch Schoenlein): immune complex deposition • Scurvy • Elhers danlos Perivascular amyloidosis
72
what is the definition of platelet deficiency
Defined as less than <150x 10(9/L- THROMBOCYTOPENIA
73
what can platelet deficiency arise from
• Decreased production: leukaemias, drug and alcohol • Decreased survival: autoimmune • Sequestration: hypersplenism( WBC taken into the spleen and destroyed Dilution: blood transfusions
74
how can we get abnormal coagulation
• vWF disease( AUTOSOMAL DOMINANT) | Haemophilia A + B- which is deficiency/lacking of factor VIII + Factor IX retrospectively
75
what is lacking in haemophilia A
factor VIII
76
what is lacking in haemophilia B
factor IX
77
describe VWdisease
``` There are 4 types: T1- 80% T2- 20% dsyfuction T3- No vWF at all T4: not compatible with life autosomal dominant disease ```
78
what is the treatment for VW disease
Desmopressin--> releases vWF OR Plasma concentrate infusion
79
describe haemophilia A
factor VIII mutations X linked recessive easy bruising/haemorrhage post surgery
80
what is the treatment for haemophilia A
Infusions of FVIII
81
describe haemophilia B
• Factor IX deficiency • X linked recessive Clinically hard to distinguish from haemophilia A
82
what is the treatment of haemophilia B
Recombinant factor IX
83
describe Disseminated intravascular coagulation(DIC)
→ It is a thrombohemorrhagic disorder → Excessive and widespread activation of coagulation → Thrombotic occlusion of small and midsized vessels → NOT A DIAGNOSIS- NEED TO FIND UNDERLYING CAUSE

Y3- I & W (21 decks)

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