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Semester 2 Case Studies > Yaffas notes for Case 7 > Flashcards

Yaffas notes for Case 7 Flashcards

1
Q

what do all blood cells begin their life as and where

A

pluripotential haemopooietic stem cells (PHSC) and in the bone marrow

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2
Q

what is a committed cell that produces erythrocytes called

A

CFU-E

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3
Q

what cells give rise to granulocytes, erythrocytes, monocytes and megakaryocytes

A

CFU-GEMM

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4
Q

what do PHSCs present with on their surface

A

CD34+ and CD38-

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5
Q

what does the stem cell factor do. SCF

A

synergises with IL-3 and GM-CSF to increase proliferation of stem cells

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6
Q

what is GM-CSF necessary for

A

growth and development of granulocyte and macrophage progenitor cells

Stimulates myeloblasts and monoblasts

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7
Q

what does G-CSF act on

A

acts on precursor cells which give rise to neutrophils

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8
Q

what does M-CSF play a role in

A

proliferation and differentiation of haematpoietic stem cells to produce monocytes and macrophages

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9
Q

what does IL-3 do

A

works in conjunction with GM-CSF to proliferate most of the haematopoietc progenitor cells

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10
Q

what does IL5 do and what produces it

A

produced by T lymphocytes and plays a role in growth and differentiation of eosinophils

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11
Q

where is thrombopoietin produced

A

mainly in the liver

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12
Q

what does thrombopoietin stimulate

A

megakaryocytes and platelet production

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13
Q

differentiation inducers factors

A

PU.1

GATA.1

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14
Q

what does PU.1 cause

A

differentiation of cells along the myeloid lineage

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15
Q

what does GATA.1 cause

A

differentiation of cells along the eryhtropoeitc and megakaryocytic lineages

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16
Q

thickness and diameter of RBCs

A

7.8 micrometers in diameter and 2.5 micrometers in thickness

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17
Q

what is the average volume of a red blood cell

A

90-95cm cubed

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18
Q

what is the normal range of the mean corpuscular volume

A

80-99fL

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19
Q

where are erythrocytes produced in early weeks of embryonic life

A

in the yolk sac

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20
Q

where are erythrocytes produced in the middle trimester of gestation

A

liver (mainly), spleen and lymph nodes

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21
Q

where are erythrocytes produced in the last month of gestation and after birth

A

RBC’s produced in the bone marrow

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22
Q

first cell that can be identified as belonging to the red blood cells series:

A

the pro erythroblast

this is formed from the CFU-E stem cells

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23
Q

how much haemoglobin do proerythroblasts have

A

very little haemoglobin

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24
Q

what forms polychromatophil and orthochromatic erythroblasts

A

basophil erythroblasts

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25
Q

what comes after the orthochromatic erythroblast:

A

reticulocyte

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26
Q

how long does it take to reach the reticulocyte stage of the RBC

A

5 days

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27
Q

final cell produced

A

mature erythrocyte - takes 1 to 2 days

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28
Q

where is erythropoietin produced

A

the kidney

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29
Q

what is erythropoietin produced as a response to

A

low tissue oxygenation

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30
Q

what stimulates erythropoietin production

A

noradrenaline and adrenaline

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31
Q

what does erythropoeitn stimulate

A

production of proerythroblasts from hematopoietic stem cells

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32
Q

life span of a RBC

A

120 days

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33
Q

what happens when a RBC goes past the 120 days

A

it becomes more fragile and cell ruptures during passage through the red pulp of the spleen. the content of the red blood cell (haemoglobin) is released and is phagocytose by the macrophages in many parts of the body.

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34
Q

what cell phagocytoses RBCs

A

Kupffer cells of the liver and macrophages of the spleen and the bone marrow

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35
Q

what does this cause

A

release of Fe into the blood

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36
Q

what carries Fe to the bone marrow for production of new erythrocytes

A

transferrin

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37
Q

Fe can be carried to the bone marrow, but where else?

A

the liver

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38
Q

how Is iron stored In the liver

A

as ferritin

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39
Q

what happens to the porphyrin portion of the haemoglobin molecule

A

converted by the macrophages to bilirubin, which is released into the blood and later removed form the body by secretion through the liver into bile

40
Q

diagram of Haematopoeisis

A
41
Q

average amount of haemoglobin per 100ml of cells in men

A

15g of haemoglobin per 100mls

42
Q

average amount of haemoglobin per 100mls in females

A

14g of haemoglobin per 100mls

43
Q

how much oxygen is each gram of haemoglobin able to combine with

A

1.34mls of oxygen

44
Q

chains that combine to form haemoglobin

A

2 alpha chains and 2 beta chains

45
Q

how much oxygen can be transported by each haemoglobin molecule and why

A

four molecules

each haemoglobin chain has a heme group containing one atom of iron and because there are four haemoglobin chains (alpha and beta) in each haemoglobin molecule, one find four iron atoms in each haemoglobin molecule, each of these can loosely bind with one molecule of oxygen making a total of four molecules of oxygen being transported by each haemoglobin molecule

46
Q

HbA occurs in what percentage of adults (normal and most common)

A

97%

47
Q

what does 2,3-DPG do

A

binds to haemoglobin molecule and lowers its oxygen affinity

48
Q

what does the liver secrete into the bile

A

apotransferrin which flows through the bile duct into the duodenum where it enters the duodenal circulation

49
Q

what does the binding of apotransferrin and free iron in the duodenal circulation form

A

transferrin

50
Q

where is transferrin transported

A

either to the liver or bone marrow

51
Q

what happens to transferrin in the liver

A

enters the hepatocytes and combines with apoferritin forming ferritin

52
Q

what happens to transferrin in the bone marrow

A

binds to receptors on the eryhtroblasts, delivering iron to the mitochondria for the production of haemoglobin

53
Q

granulopoiesis

A
  • myeoblasts of varying size, large nucleus and no cytoplasmic granules are the first committed cells
54
Q

what do myeoblasts form

A

promyelocytes which comprise of primary cytoplasmic granules

55
Q

what do promyelocytes form

A

myelocytes depending on the cell type

56
Q

what do myelocytes form

A

metamyelocytes which are non-dividing cells with an indented nucleus and many cytoplasmic granules

57
Q

what percentage of the circulating leucocytes are neutrophils

A

40-75%

58
Q

how many lobes do mature leucocytes have

A

5 lobes

59
Q

eosinophils constitute what percentage of leucocytes

A

1-6%

60
Q

what percentage of leucocytes do basophils consitute

A

less than 15

61
Q

what percentage of leucocytes do basophils consitute

A

less than 1%

62
Q

monopoieses timeline

A
  1. monoblast is first committed cell
  2. this divides and differentiate into a promonocyte
  3. this differentiates into a mature monocyte
63
Q

percentage of leucocytes that monocytes constitute

A

2-10%

64
Q

how long do monocytes circulate for before turning into macrophages in tissues

A

2-4 days

65
Q

where does lymphopoeiss occur in a foetus and an adult

A

the liver in a foetus

the bone marrow in an adult

66
Q

what do B lymphocytes mature into

A

plasma cells

67
Q

where are plasma cells formed

A

in the lymph nodes and themselves produce antibodies

68
Q

where to T Lymphocytes mature

A

in the thymus

69
Q

percentage of circulating leucocytes that lymphocytes make up

A

20-50%

70
Q

3 reasons for anaemia

A
  1. reduced production of RBCs
  2. increased loss of RBCs
  3. haemoglobinopathies: sickle cell anaemia and thalassaemia
71
Q

what is macrocytic anaemia a result of

A

vitamin B12 deficiency, folate deficiency and alcohol excess (liver disease)

72
Q

what is normocytic anaemia a result of

A

acute blood loss, anaemia of chronic disease, pregnancy and bone marrow failure/supression

73
Q

what is microcytic anaemia a result of

A

iron-deficiency anaemia or thalassaemia

74
Q

reduced production of RBCs is a result of what deficiencies

A

iron deficiency
folate deficiency
Vitamin B12 deficiency

75
Q

what is reduced production of RBCs a result of (non-deficiencies)

A

anaemia of chronic disease (rheumatoid arthritis - decreases folate)
cancers e.g leukaemia

76
Q

increased destruction and loss of RBCs is a result of

A
  • blood loss
  • sickle cell disease
  • thalassaemia
  • erythrocyte membrane defects
  • cancers
  • haemolytic disease of a newborn
  • incompatible blood transfusion
77
Q

blood film appearances

A
78
Q

treatment for iron deficiency anaemia

A

ferrous sulphate

79
Q

what is vitamin B12 anaemia caused by

A

malabsorption of the vitamin

80
Q

what is pernicious anaemia

A

term used to describe vitamin B12 deficiency resulting from the inadequate gastric production or defective function of intrinsic factor

81
Q

treatment for pernicious anaemia

A

folate

intramuscular vitaminB12

82
Q

causes of folate deficieny anaemia

A
  • diet/decreased intake
  • increased requirements
  • impaired utilisation
83
Q

treatment for folic deficiency anaemia

A

oral folic acid

84
Q

two haemoglobinopathies

A

sickle cell disease

thalassaemia

85
Q

what is the genetic variation in sickle cell disease

A

genetic variation in the beta global chain of the Hb molecule (HbS)

86
Q

what happens when a person has sickle cell disease

A

the Hb molecule becomes unstable in low oxygen condition leading to the formation insoluble rigid chains
this causes vasoconstriction-occlusion and destruction of the red cell - haemolysis

87
Q

pathogenesis of sickle cell disease

A

HbS molecules undergo polymerization (combine) when deoxygenated.
Initially the red cell cytosol converts from a freely flowing liquid to a viscous gel as HbS aggregates form.
With continued deoxygenation aggregated HbS molecules assemble into long needle-like fibres within red cells, producing a distorted sickle shape.
The presence of HbS underlies the major pathologic manifestations:
Chronic haemolysis
Microvascular occlusions
Sickle red cells express higher than normal levels of adhesion molecules and are sticky.
Tissue damage: this is as a result of the microvascular occlusions causing certain tissue to become hypoxic as a result of ischemia.

88
Q

clinical features of SCA

A 
anaemia 
stroke 
acute chest syndrome 
impaired growth and development 
chronic organ damages
89
Q

treatment for sickle cell anaemia

A

bone marrow transplants
prophylactic antibiotics due to increased susceptibility of infections
blood transfusion to reduce the amount of HbS cells

90
Q

what is thalassaemia

A

a group of inherited disorders resulting in reduced production of one or more global chains.
this results in an imbalance of globin chains ad many pathological effects

91
Q

pathological effects of thalassaemia

A
  • damage to red cell precursors - leading to ineffective eryhtropoeisis
  • damage to mature blood cells leading to haemolytic anaemia
92
Q

what type of anaemia does thalassamia mainly cause

A

microcytic anaemia

93
Q

what are the two main types of thalassaemia

A

alpha thalasseamia

beta thalassaemia

94
Q

clinical classification of thalassaemia:

A 
- Thalassaemia Major
Continual transfusion dependent 
- Thalassaemia Intermedia
Less severe anaemia and can survive without regular blood transfusions
- Thalassaemia Minor/ Carrier
Asymptomatic carrier
95
Q

clinical features of thalassaemia

A
  • problems due to anaemia e.g failure to grow and develop

- problems due to iron overload - failure of grow and mature

96
Q

blood group table

A

Semester 2 Case Studies (11 decks)

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