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Haematology > Week 1 > Flashcards

Week 1 Flashcards

1
Q

What is the name for the production of blood cells?

A

Haemopoiesis

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2
Q

Where are the sites of haemotpoiesis for an embryo?

A

Yolk sac

between 12wks - 28wks

spleen

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3
Q

Where are the sites of heamtopoiesis in an adult?

A

axial skeleton i.e

skull

ribs

sternums

pelvis

proximal limbs

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4
Q

What is the name for the making of red blood cells?

A

Erythropoiesis

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5
Q

What are the cells at each stage of erythropoiesis called?

A

Pronormoblast

Early normoblast

Intermediate Normoblast

Late normoblast

Reticulocyte

Erythrocyte

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6
Q

What are the three types of granulocytes?

A

Eosinophils

Basophils

Neutrophils

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7
Q

What do neutrophils do?

A
  • Short life
  • Phagocytose invaders
    • Up regulate inflamation
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8
Q

What do neutrophils look like?

A

Polymorph segmented nucleus

Neutral staining granules

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9
Q

What do eosinophils do?

A

Bi lobed nucleus with orange/red granules

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10
Q

What do Eosinophils do?

A

Fight parasites

hypersensitivity

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11
Q

what are monocytes?

A

Monocytes live for weeks in the blood and migrate into the tissues to become macrophages

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12
Q

Where are bone marrow samples usually taken from?

A

posterior iliiac crest

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13
Q

What options are there for taking bone marrow samples?

A

Vaccum aspiration

Core biopsy

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14
Q

What are the properites of mature red blood cells?

A

Packe with haemoglobin

No nucleus, mitochondria

Life span = 120days

energy from glycolosis

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15
Q

What happens to old red blood cells?

A

Broken down by the spleen

Haem group becomes bilirubin

Iron binds to transferrin and is recycled

Proteins become amino acids and are recycled

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16
Q

What hormone regulates erythrocyte production?

A

erythropoietin

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17
Q

What stimulates the relase of erythropoietin?

A

Reduced oxygen carrying capacity

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18
Q

What are the ratios of plasma, white blood cells , platelets and RBC in blood?

A

55% plasma

54% Erythrocytes

1% white blood cells and platelets

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19
Q

What do red blood cells need energy for?

A

maintaining ion balance and cell volume with Na+/K+ ATPase pump

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20
Q

What other benefit does making NADH have for red blood cells ( other than the energy)

A

it helps keep iron in its Fe 2+ state

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21
Q

What is used by the body to combat oxidative stress?

A

Glutathione

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22
Q

Where does glutathione come from?

A

Comes from the hexose monophosphate shunt

an alternative way of metabolising glucose

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23
Q

Wha makes up glutothione?

A

Glutamate

Cysteine

glycine

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24
Q

What are the three ways CO2 is carried to the lungs?

A
  1. Dissolved in soloution - 10%
  2. Bound to Hb - 30%
  3. As bicarbonate ion HCO3- 60%
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25
Q

What enzyme helps CO2 to become the bicarbonate ion?

A

Carbonic anhydrase

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26
Q

What is the structure of the adult haemoglobin?

A

4 globin protein sub-units each containing a single haem molecule

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27
Q

What is the fuction of the haem group?

A

Each haem group contains on Fe 2+ ion and can bind to one O2 molecule

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28
Q

What are the normal haemoglobin conentrations in adults?

A

130-180 g/L in males

115-165 g/L i females

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29
Q

Why can oxygen be transferred from the mothers blood to the foetal blood?

A

Foetal haemoglobin has a higher affinety for O2 than adults

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30
Q

Why can oxygen be transferred from your blood to your muscles?

A

Myoglobin has a greater affinity for oxygen than CO2

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31
Q

Why is it so important that red blood cells have a working ion pump?

A

As they are stuffed with Hb they have a high osmotic pressure.

They need the pump to stop them bursting

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32
Q

What enables red blood cells to have such a weird shape?

A

The membrane is protein rich so that it is stretchy and cool

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33
Q

What pathway regenerates glutathione?

A

Hexose monophosphate shunt

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34
Q

what does anaemia mean?

A

Reduced total red cell mass

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35
Q

What is haematocrit?

A

Percentages of whole blood that is made up of red bloods cels

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36
Q

How is Hb measured?

A

burst the cells and shine a light through them to see how red they are

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37
Q

In what situations are Hb/Hct not very useful for measuring aneamia?

A
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38
Q

What is the bodies response to anaemia?

A

Increased red cell production

Reticulocytosis

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39
Q

What are reticulocytes?

A

Red cells that have just left the bone marrow

they are big and still have some RNA

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40
Q

How long does it take for the up regulation of reticulocyte production in response to aneamia to have an affect?

A

a few days

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41
Q

What is MCV?

A

Mean cell volume

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42
Q

What does low MCV suggest?

A

Problem with haemoglobinisation

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43
Q

What does high MCV suggest?

A

Problem with maturation

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44
Q

What causes microcytic aneamia?

A

Failed heamaglobin synthesis

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45
Q

What is required to make Hb?

A

Globins

Haem - porphyrin ring and iron

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46
Q

What do microcytic aneamic red blood cells look like?

A

Real small

hypochromic

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47
Q

How much iron is absorbed per day?

A

1mg/day

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48
Q

What is important to remember about iron in the body?

A

Its a closed system

most of the iron is recycled

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49
Q

in what forms is iron transferred and stored?

A

circulating iron is bound to transferrin

iron is stored as ferritin in the liver

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50
Q

What is transferrin?

A

Protein with two binding sites for iron

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51
Q

What does transferrin do?

A

Transports iron from donor tissuses ( intestinal cells) to erythroid marrow

52
Q

What does percentage saturation of transferrin give you information about?

A

Iron supply

53
Q

What is the most common cause of iron deficency?

A

Dietry

54
Q

What hand sign indicateds low iron levels

A

Koilnonychia

55
Q

What is marcocytic anaemia?

A

Anaemia in which the red cells have a larger than normal volume

56
Q

What does macrocytosis mean?

A

Large cells and lots of them

57
Q

What is the normal mean corpuscular volume of red blood cells?

A

80-100 femtolitres

58
Q

What is a megaloblast?

A

An abnormally large nucleated red cell precursos with an immature nucleus

59
Q

What are the over arching causes of megaloblastic anaemias?

A

Defects in DNA synthesis and Nuclear maturation

60
Q

What can cause megaloblastic anaemia?

A

B12 deficiency

Folate deficiency

Rare genetic shit

drugs

61
Q

Why does lack of B12 or Folate cause megaloblastic anaemia?

A

They are esential for nuclear maturation.

Need them for DNA synthesis

62
Q

What is pernicious anaemia?

A

Autoimmune condition destorying gastric parietal cells.

Intrinsic factor deficiency an B12 malabsorption

63
Q

Where is folate absorbed ?

A

In the jejunum

64
Q

How long do your body stores of

Folate

B12

last?

A

Folate - 4 months

B12- 2-4 yrs

65
Q

Where is B12 absorbed

A

Ileum

66
Q

What are the excess utilisation causes of folate deficiency?

A

Haemolysis

Exfoliating dermatitis

Pregnancy

Malignancy

anti convulsant medication

67
Q

What does B12 deficency cause?

A

Aneamia type symptoms

plus

Neurlogical problems

68
Q

What neurological problems does B12 deficiency cause?

A

Posterior/dorsal column abnormalities

69
Q

what does pancytopenia?

A

Low levels of all cells

70
Q

What can you use to measure B12 and Folate?

A

assay B12 and folate levels in serum

71
Q

How do you treat pernicious anaemia?

A

life long vitamin B12 injections

72
Q

What are the causes of non-megaloblastic macrocytosis?

A

Alcohol

Liver disease

Hypothyroidisim

Marrow failure

73
Q

What is spurious macrocytosis?

A

Size of mature red cell is normal but the MCV is measured as being high

74
Q

What causes spurious macrocytosis?

A

Increase in reticulocytes

(reticulocytes are bigger)

if blood cells cool and clump together

75
Q

What are haemoglobinopathies?

A

Hereditary conditions affecting globin chain synthesis

76
Q

Whats the difference between

HbA

HbA2

HbF

A

HbA - 2 alpha 2 beta

HbA2- 2 alpha 2 delta

HbF- 2 alpha 2 gamma

77
Q

What percentage of each type of heamglobin is found in adults?

A

HbA - 97%

HbA2 - 2.5%

HbF - 0.5%

78
Q

What is alpha thalassaemia trait?

A

One faulty alpha chain

Microcytic, hypochromic red cells with mild anaemia

79
Q

What does structural haemoglobin variants mean?

A

Normal production rate of structurally abnormal globin chains

80
Q

What results from thalassaemia?

A

Microcytic hypochromic aneamia

81
Q

What chromosome are alpha chains coded on?

A

Coded on chromosome 16

82
Q

What is barts hydrops fetalis?

A

No functional alpha genes

83
Q

What is HbH disease?

A

Severe form of apha thalassaemia

84
Q

What happens in HbH disease?

A

Only one alpha gene per cell

aneamia with very low MCV and MCH

Also get haemglobins wit 4 beta chains that for tetramers

85
Q

How does HbH disease present?

A

Moderate aneamia

Splenomegaly

Jaundice

86
Q

Where is HbH disease most common?

A

S.E asia

Middle east

Mediterranean

87
Q

What are the three classes of beta thalassaemia?

A

Beta thalassaemia trait

Beta thalassaemia intermedia

Beta thalassaemia major

88
Q

How does beta thalassaemia major persent?

A

At 6 months of age

Pallor, failure to thrive

Hepatosplenomegaly

Skeletal changes

Organ damage

89
Q

How do you trat beta thalassaemia major?

A

Regular transfusion program to maintain Hb

This suppresses erythropoiesis of the bad stuff

90
Q

What must you be vary of in people who get regular blood transfussions?

A

Iron overload

91
Q

How do you treat iron overload?

A

Iron chelating drugs allow you to pee or poo it out

Desferrioxamine

92
Q

What are the consequences of iron overload?

A

Endocrcine- growth, diabetes, Osteoporosis

Cardiac disease- cardiacmyopathy, arrhythmias

Liver diseasep- cirrhosis, hepatocellular cancer

93
Q

What is sickle trait?

A

One normal beta gene

one abnormal beta gene

94
Q

What causes sickle cell aneamia?

A

Two abnormal copies of beta genes

95
Q

What is a sickle crisis?

A

Episodes of tisse infarction due to vascular occlusion

96
Q

What are the common symptoms of sickle cell anaemia?

A

Sickle crisis

Chronic haemolysis

Hyposplenism

97
Q

What can precipitate a sickle crisis?

A

Hypoxia

Dehydration
infection

Cold exposure

Stress/fatigue

98
Q

How do you treat sickle crisis?

A

Opiate analgease

Hydration

Red cell exchange

99
Q

How do you treat sickle cell aneamia in the long term

A

Prophylactic pencillin

Folic acid

Hydroxycarbamide

100
Q

What is haemolysis?

A

Premature red cell destruction

101
Q

What are red cells so suspetable to damage?

A

Biconconave shape required for ciculation

limited metabolic reserve

cant generate new proteins

102
Q

What is compensated haemolysis?

A

Increased red cell destruction compensated by increased red cell production

103
Q

What are the physiological consequences of haemolysis?

A

Erythroif hyperplasia

Excess red cell breakdown products

104
Q

Whats the difference between extravascular and intravascular heamolysis?

A

Extravascular - reticuloendothelium system (liver/spleen)

Intravascular- red cells destroyed within circulation

105
Q

What are the consequences of intravascular red cell destruction?

A

Haemoglobinaemia

Methaemalbuminaemia

Haemoglobinuria - pink urine, black on standing

Haemosideriunuria

106
Q

What do spherocytes of blood film suggest

A

Membrane damage

107
Q

What do red cell fragments on blood film suggest?

A

Mechanical damage

108
Q

What do heinz bodies on blood films suggest?

A

Oxidative damage

109
Q

What are the classifications of haemolyisis by site of red cell defect?

A

Premature destruction - immune or mechanical

Abnormal cell membrane

Abnormal red cell metabolisim

Abnormal haemoglobin

110
Q

What antibiodies cause Autoimmune Haemolysis?

A

IgG

IgM

111
Q

What can cause red cell membrane defects resulting in haemolysis?

A

Liver diesease
and
Vitamin E deficiency

112
Q

What is Zieve’s Syndrome?

A

Haeloytic aneamia with alcoholic liver disease

and

Polychromatic macrocytes

113
Q

What are the components that make up haem?

A

Porphyrin ring + Fe 3+ = Haem

114
Q

What transporters absord iron into the dudodenal enterocyte?

A

DMT-1

Divalent Metal transporter 1

transports iron into the dudodenal enterocytes

115
Q

What facilitates iron export from the enterocyte to transferrin?

A

Ferroportin

116
Q

What down regulates ferroportin?

A

Hepcidin

117
Q

Where is Hepcidin produced and why?

A

Produced in the liver in response to high iron levels

118
Q

Whats the difference between holotransferrin and apotransferrin?

A

Holotransferrin is transferrin with iron attatched

apotransferrin is transferrin without iron attatched

119
Q

What are the three compartments looked at in the assesment of iron status?

A

Functional iron - haemoglobin concentration

Transport iron - Transferrin saturation

Storage iron - serum ferritin

120
Q

What are globin deficiencies called?

A

Thalassaemias

121
Q

What causes haemochromatosis?

A

Mutations of the HFE gene

122
Q

How do you treat haemochromatosis?

A

Weekly venesection

123
Q

Whats the risk of haemchromatosis in first degree relatives

A

1 in 4

124
Q

What is important to remember about haemochromatosis?

A

It can be asympotmatic untill ireversible organ damage has occoured

125
Q
A

Haematology (4 decks)

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