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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
Q

if the cells are small what do we call the anaemia

A

microcytic anaemia if they are smaller than 80 femotolitres

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26
Q

if the cells are large what do we call the anaemia

A

macrocytic anaemia if they are larger than 96 femtolitres

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27
Q

what do we call the anaemia if it is between 80-96 femtolitres

A

then you have normocytic anaemia

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28
Q

describe iron deficient anaemia

A

associated with due to microcytic anaemia

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29
Q

what are the cuases of iron deficient anaemia

A

blood loss
increased demand eg pregnancy
decreased absroption eg surgery
poor dietary intake

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30
Q

how do we investigate iron deficient anaemia

A

blood count
blood film
iron status

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31
Q

what do we expect to see on the blood count of someone with iron deficient anaemia

A

microcytic anaemia

hypochromic anaemia

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32
Q

what do we expect to see on the blood film of someone with iron deficient anaemia

A

poikilocytosis- change in shape

anisocytosis- variation in size

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33
Q

what are other causes of iron deficient anaemia

A

anaemia chronic disease
thalassaemia
sideroblastic anaemia

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34
Q

Describe megaloblastic anaemia

A

associated with macrocytic anaemia

associated with vitamin B12

35
Q

how much vitamin b12 do we need a day

A

2-3micrograms/day

36
Q

how much vit b12 can we take in a day

A

20 micrograms if we eat meat/eggs and it is stored in the liver

37
Q

how do we absorb vitamin b12

A

by IF in the small bowel

binds to IFb12 complex absorbed in the small bowel

38
Q

what is IF secreted by

A

gastric parietal cells

39
Q

the process of vitamin b12 absorption

A
  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
Q

what is pernicious anaemia

A

autoimmune condition
due to the loss of parietal cells in the stomach
less IF produced therefore less B12 absorption

41
Q

what do we see in the blood count of pernicious anaemia

A

large MCV macrocytic anaemia

42
Q

what do we see in the blood film of pernicious anaemia

A

oval macrocytes
hyper segmented polymorphs
severe deficiency- leukopenia/thrombocytopenia

43
Q

what do we see in the bone marrow of pernicious anaemia

A

hypersegmented polymorphs

increased megaloblasts

44
Q

what is the treatment of pernicious anaemia

A

IM b12 injections

45
Q

why does b12 deficiency/ folate deficiency cause macrocytic anaemia

A

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
Q

what are the causes of folate deficiency

A

malnutrition
malabsorption
increased demand
some drugs- anti-epileptics

47
Q

give an example of normocytic anaemia

A

haemolytic anaemia

48
Q

describe haemolytic anaemia

A

the RBCS have a short life span

it is an acquired disease

49
Q

what ate the different types of haemolytic anaemia

A

haemoglobin synthesis abnormalities
red cell membrane defects
metabolic defects

50
Q

give an example of anaemia that occurs from haemoglobin synthesis abnormalities

A

sickle cell anaemia

51
Q

give an example of anaemia that occurs from red cell membrane defects

A

hereditary spherocytosis

52
Q

give an example of anaemia that occurs from metabolic defects

A

Glucose-6 phosphate dehydrogenase deficiency

53
Q

describe sickle cell anaemia

A

involves a single gene substitution mutation

where valine is subed for glutamic acid on the 6th codon of the beta globin chain

54
Q

what happens when there is low levels of o2 in people with sickle cell anaemia

A

the HbS molecule sticks together

55
Q

how can the sickle shape occur

A 
Infection 
	Dehydration 
	Acidosis 
	Hypoxia
Cold
56
Q

what does sickle cell anaemia result in

A

haemolysis

obstruction of the small vessels

57
Q

clinical features of sickle cell anaemia

A

· 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
Q

what are the symptoms of sickle cell anaemia

A

shortness of breath
chest pain
hypoxia

59
Q

investigaations of sickle cell anaemia

A

blood count- normocytic anaemia

blood film: sickled red cells

60
Q

treatment of sickle cell anaemia

A

depends on the complications

61
Q

oral manifestations of anaemia

A

angular chelitis
atrophic glossitis
recurrent apthous stomatitis

62
Q

what is angular chelitis

A

soreness of the corners of the mouth

63
Q

what is atrophic glossitis

A

smooth tongue

64
Q

what is recurrent apthous stomatitis

A

ulcers in the mouth for 10-14 days
seen with b12/folate, iron deficiencies
check the haematinics

65
Q

what is haemostasis

A

blood clotting at a site of injury

66
Q

what is the sequence of blood clotting

A
  1. vasoconstriction
  2. platlet plug formation
  3. fibrin meshwork
  4. insoluble firinogenin meshwork
67
Q

what happens in vasoconstriction

A

Reduces the blood flow
Endothelin is released which leads to constriction of the blood vessels
It is a temporary fix

68
Q

what happens in platelet plug formation

A

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
Q

what happens in fibrin meshwork

A

The Tissue factor is exposed
Activates the coagulation cascade
Thrombin–> cleaves fibrinogen–> insoluble fibrin and then meshwork
RBC entrapment

70
Q

how can bleeding disorders occur

A 
from defects in the 
vessel wall abnormalities 
platelet deficiency
abnormal coagulation 
acquired deficiencies
liver disease
71
Q

describe vessel wall abnormalities

A

• Can be from infection eg meningococcal septicaemia
• Drug reactions( Henoch Schoenlein): immune complex deposition
• Scurvy
• Elhers danlos
Perivascular amyloidosis

72
Q

what is the definition of platelet deficiency

A

Defined as less than <150x 10(9/L- THROMBOCYTOPENIA

73
Q

what can platelet deficiency arise from

A

• Decreased production: leukaemias, drug and alcohol
• Decreased survival: autoimmune
• Sequestration: hypersplenism( WBC taken into the spleen and destroyed
Dilution: blood transfusions

74
Q

how can we get abnormal coagulation

A

• vWF disease( AUTOSOMAL DOMINANT)

Haemophilia A + B- which is deficiency/lacking of factor VIII + Factor IX retrospectively

75
Q

what is lacking in haemophilia A

A

factor VIII

76
Q

what is lacking in haemophilia B

A

factor IX

77
Q

describe VWdisease

A 
There are 4 types: 
	T1- 80%
	T2- 20% dsyfuction 
	T3- No vWF at all
T4: not compatible with life 
autosomal dominant disease
78
Q

what is the treatment for VW disease

A

Desmopressin–> releases vWF
OR
Plasma concentrate infusion

79
Q

describe haemophilia A

A

factor VIII mutations
X linked recessive
easy bruising/haemorrhage post surgery

80
Q

what is the treatment for haemophilia A

A

Infusions of FVIII

81
Q

describe haemophilia B

A

• Factor IX deficiency
• X linked recessive
Clinically hard to distinguish from haemophilia A

82
Q

what is the treatment of haemophilia B

A

Recombinant factor IX

83
Q

describe Disseminated intravascular coagulation(DIC)

A

→ 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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