haematological diseases Flashcards

1
Q

what is this abbreviation FBC

A

full blood count which includes all of the values below
red blood cells, white blood cells, platelets, haematocrit, mean cell volume

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2
Q

what is the abbreviation RBC

A

red blood cells or RCC red cell count

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3
Q

what is the abbreviation WCC

A

white cell count

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4
Q

what is the abbreviation PLT

A

platelets

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5
Q

what is the abbreviation

A

haematocrit

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6
Q

what is haematocrit

A

ratio of cells to liquid in the blood

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7
Q

what is the abbreviation MCV

A

mean cell volume - volume of the average red blood cell

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8
Q

what blood change is in anaemia ?

A

low haemoglobin

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9
Q

what blood change is in leukopenia

A

low WCC

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10
Q

what blood change is in thrombocytopenia?

A

low platelets

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11
Q

what blood change is in pancytopenia?

A

all cells reduced

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12
Q

one of theses change in the blood is due to what most often?

anaemia
leukopenia
thrombocytopenia
pancytopenia

A

environment

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13
Q

multiple of these changes in the blood is due to what most often?
anaemia
leukopenia
thrombocytopenia
pancytopenia

A

bone marrow failure

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14
Q

what blood change is in polycythaemia?

A

raised haemoglobin (opposite of anaemia)

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15
Q

what blood change is in leukocytosis?

A

raised WCC (normal if fighting infection)

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16
Q

what blood change is in thrombocythaemia?

A

raised platelets - can be a reactive change to damage in the body

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17
Q

one of these changes in the blood is what?

polycythaemia
leukocytosis
thrombocythaemia

A

reactive or pre-neoplastic

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18
Q

multiple of these changes in the blood is likely what?

polycythaemia
leukocytosis
thrombocythaemia

A

pre-neoplastic, myelodysplasia precancerous

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19
Q

the myeloid stem cell group produces what cells? 5

A

○ Red cells
○ Platelets
○ Neutrophils
○ Basophils
○ Eosinophils
Monocytes

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20
Q

the lymphoid stem cell group produces what cells? 3

A

B cells
T cells
NK cells

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21
Q

Blood cancer can derive from either the lymphoid or the myeloid cell line and this determines its ?

A

behaviour

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22
Q

definition of leukaemia

A

Neoplastic proliferation of white cells, usually disseminated

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23
Q

definition of lymphoma

A

○ Neoplastic proliferation of white cells, usually a solid tumour

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24
Q

what is porphyria?

A
  • Abnormality of haem metabolism
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25
Q
  • Acute intermittent porphyria’s can be triggered by ? including ?
A

medicines
LA

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26
Q

clinical effect of porphyria? 3

A

Photosensitive rash

Neuropsychiatric disturbance in acute attacks

Hypertension and tachycardia

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27
Q

how do u prevent porphyria in pt?

A

find out trigger

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28
Q

3 causes of anaemia

A

reduced production
increased losses
increased demand

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29
Q

2 causes of reduced production

A

Reduction in the number of normal blood cells due to marrow failure

Normal blood cells but reduced haemoglobin

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30
Q

2 causes of normal blood cells but reduced haemoglobin

A

haematinic deficiency - iron (ferritin), folate, vit B12

Abnormal globin chains that can’t be manufactured into haemoglobin

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31
Q

what are the haematinics? 3

A

things used to produce red cells
iron - ferritin
folate
Vit b12

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32
Q

Iron absorption

Haem iron absorbed in a ?? in the ? wall

Non-heme iron has to go through a conversion of ? to ? then through a ? in the small intestine wall

Therefore simpler for body to deal with ? iron than ? iron
Once iron in the cell it is stored as ?, which is then passed out into the ? and carried away for ?

A

heme transporter
small intestine

Fe3+
Fe2+
transporter

heme
non-heme
ferritin
blood
recirculation

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33
Q

name 2 diseases that cause reduced iron absorption

A

achlorhydria
coeliac disease affecting the small intestine

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34
Q

how does achlorhydria reduce iron absorption?

A

lack of stomach acid stops iron conversion to haem iron

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35
Q

name 4 diseases that cause iron loss

A

Gastric erosions and ulcers

Inflammatory bowel disease - Crohns and uclerative colitis

Bowel cancer - colon and rectal

Haemorrhoids

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36
Q

causes for folate deficiency? 2

A

Lack of intake

Absorption failure - jejunal disease - coeliac disease

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37
Q

folate deficiency can cause what in a foetus?

A

neural tube defects

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38
Q

Absorption of vit b12

? cell secrete ? which binds to Vitamin B12. absorbed in the ? through a specific ?.

Problem with intrinsic factor secretion, dietary B12 or disease of the terminal ileum then absorption of vitamin B12 orally isn’t possible. Then ? are given.

A

parietal
intrinsic factor
ileum
transporter

injections

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39
Q

causes of deficiency of vitamin B12? 3

A

Dietary lack

Lack of intrinsic factor - pernicious anaemia, gastric disease

Disease of terminal ileum - Crohn’s disease

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40
Q

what 2 things can cause abnormal global chains that can’t be manufactured into haemoglobin?

A

thalassaemia
sickle cell anaemia

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41
Q

clinical affects of thalassaemia? 5

A

Chronic anaemia

Marrow hyperplasia - bigger to make more haemoglobin

Splenomegaly - spleen recycles RBCs as they are abnormal so have shorter lifespans

Cirrhosis - excess iron

Gall stones - haem reprocessing causes more chemicals to pass the liver

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42
Q

management of thalassaemia 2

A

Blood transfusion of normal RBCs - issue of iron overload - cirrhosis

Prevent iron overload risk from blood transfusions

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43
Q

what is sickle cells anaemia?

A

Abnormal globin chains that function normally at standard O2 environments but at low O2 environments change shape which prevents RBC passing through capillaries, which blocks the circulation causing ischaemia to the tissue

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44
Q

The size of the red blood cells (MCV) can show the cause of the anaemia

Raised cell volume indicates what cause?

A

vit B12 or folic acid deficiency

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45
Q

The size of the red blood cells (MCV) can show the cause of the anaemia

small red blood cells indicated what cause?

A

iron deficiency or thalassemia

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46
Q

key things to learn with anaemia

anaemia is reduced haemoglobin in the blood from the normal values of the population

haemaglobin issues can be from an inability to make haem or an inability to make the correct globin chains

anaemia is nothing to do with RBC numbers

the size of the red blood cells (MCV) can give a clue into the cause of the anaemia

A
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47
Q

anaemia diagnosis
haemoglobin levels show what?

A

the degree of anaemia

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48
Q

anaemia diagnosis
red cells count shows what?

A

if there is a normal or reduced number of red cells -> cell deficiency or haemoglobin formation deficiency

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49
Q

anaemia diagnosis
haematocrit shows what?

A

shows if there are fewer cells in the blood -> cell deficiency

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50
Q

anaemia diagnosis
mean cell volume shows what?

A

different deficiency pictures to work out the deficiency

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51
Q

anaemia with normal red blood cells is caused by what?

A

bleeding - GI bleeding

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52
Q

anaemia cell terminology - MCV

what does microcytic mean and what does it indicate the cause is?

A

small RBC
iron deficiency, Thalassemia

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53
Q

anaemia cell terminology - MCV

what does macrocytic mean and what does it indicate the cause is?

A

large RBC
B12/folate deficiency

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54
Q

anaemia cell terminology - MCV

what does normocytic mean and what does it indicate the cause is?

A

normal RBC size
bleed, renal, chronic disease

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55
Q

anaemia cell terminology

what does hypo chromic mean?

A

appear pale under microscope due to less haemoglobin

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56
Q

anaemia cell terminology

what does ansiocytic mean?

A

very big cells and very small cells

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57
Q

what are reticulocytes?

A

Almost mature RBCs released early into circulation to replace losses

They still have organelles so are larger and raise the MCV

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58
Q

anaemia signs and symptoms 7

A

Signs
Pale
Tachycardia
Enlarged liver/spleen

Symptoms
Tired and weak
Dizzy
Shortness of breath
Palpitations due to high heart rate

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59
Q

dental signs of anaemia? 4

A

pale mucosa
smooth tongue
angular cheilitis
beefy tongue

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60
Q

smooth tongue is a sign of what haemotinic deficiency?

A

iron deficiency

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61
Q

angular cheilitis is a sign of what haemotinic deficiency?

A

iron deficiency

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62
Q

beefy tongue is a sign of what haemotinic deficiency?

A

vit B12 deficiency

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63
Q

what investigations are done for anaemia? 5

A

full blood count - ferritin, folate, vitamin b12

GI blood loss causing anaemia
- faecal occult blood test
- endoscopy

renal function

bone marrow examination

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64
Q

what is the treatment for anaemia? 4

A

treat the cause

replace haematinics - iron, folate, vit b12

transfusions for production failure

erythropoietin if production failure due to renal disease (kidneys)

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65
Q

how is the haemotinic iron replaced?

A

FeSO4 200mg for 3 months

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66
Q

how is the haemotinic vit B12 replaced?

A

1mg IM vit B12 x 6 then 1mg/2months

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67
Q

how is the haemotinic folate replaced?

A

5mg folic acid daily

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68
Q

How to differentiate deficiency from bleeding

If bleed RCC ?, MCV is ?, HCT is ?

Deficiency RCC is ?, MCV is ? or ?, HCT is ?

A

low
normal
low

low
micro
macro-cytic
normal

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69
Q

normal haemoglobin range in male and female

A

male 130-180, female 115-165 g/litre

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70
Q

normal RCC range in male and female

A

male 4.5-6.5, female 3.8-5.8 x 1012/litre

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71
Q

normal WCC range

A

3.6-11 x 109/litre

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72
Q

normal MCV range

A

80-100 fl

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73
Q

normal haematocrit range

A

male 0.4-0.54, female 0.37-0.47 L/L

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74
Q

normal PLT range

A

140-400 x 109/L

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75
Q

what are the ferritin levels in iron deficiency anaemia?

A

<30 micrograms/litre

76
Q

folate/vitamin B12 anaemia results in micro or macro cytic anaemia?

A

macrocytic anaemia -> large RBCs

77
Q

common causes of blood loss that leads to anaemia? 5

A

Ulcers
Polyps
Cancers in large intestine - colon, rectal
Kidney/bladder tumours
Menstrual bleeding

78
Q

name 2 types of anti thrombotic medication

A

Injectable anticoagulants
oral anticoagulants

79
Q

are injectable anticoagulants a dental issue?

A

no

80
Q

are oral anticoagulants a dental issue?

A

yes

81
Q

oral anticoagulants interfere with what?

A

the clotting cascade

82
Q

what are the two types of oral anticoagulation?

A

coumarin and non-coumarin

83
Q

is warfarin a coumarin or non-coumarin?

A

coumarin

84
Q

mechanism of action for warfarin

A

vitamin K antagonist - slow onset, initially hypercoagulable

85
Q

warfarin

treat normally if INR below ?, delay treatment if above ?
INR measured within ?, preferably ?, prior to treatment

A

4
4
72hrs
24hrs

86
Q

drugs to avoid prescribing if pt taking warfarin 3

A

Aspirin, NSAIDS
Azole antifungal drugs

87
Q

name a novel oral anticoagulant

A

apixaban

88
Q

NOAC apixaban mechanism of action

A

Factor Xa inhibitor

89
Q

for the NOAC apixaban what should u do for low risk procedures? 3

A

don’t interrupt medication
treat early in morning
treat in stages

90
Q

for the NOAC apixaban what should u do for higher risk procedures? 2

A

miss/delay morning dose, restart after treatment

91
Q

do NOACs require monitoring?

A

no

92
Q

how often should a pt take apixaban?

A

twice daily

93
Q

drugs to avoid prescribing for NOACs 3

A

NSAIDs

Macrolide antibiotics -> erythromycin and clarithromycin

Carbamazepine

94
Q

mechanism of action for anti platelet medication

A

○ Interfere with platelet number or function

95
Q

name 2 antiplatelet medications

A

aspirin
clopidogrel

96
Q

should u interrupt anti platelet medications for treatment

A

no but treat in stages

97
Q

If two antiplatelet drugs are taken without aspirin then speak to doctor to stop one ? days prior to surgery

A

7

98
Q

drugs to avoid prescribing if pt on antiplatelet medication 6

A

NSAIDs
Erythromycin
Fluconazole
Clarithromycin
Carbamazepine
Omeprazole

99
Q

indications for anticoagulation requirement

A

Atrial fibrillation
Deep venous thrombosis
Heart valve disease
Mechanical heart valves
Thrombophilia

100
Q

inherited bleeding disorder is an acquired defect that affects what?

A

the coagulation of the blood

101
Q

what three things can inherited bleeding disorders affect?

A

coagulation cascade
platelets
a combined deficiency

102
Q

how can bleeding disorders affect the coagulation cascade

A

reduction in one or more of the coagulation factors or control proteins

103
Q

what is haemophilia?

A

too much clot formed

104
Q

does haemophilia affect males or females?

A

males as on X chromosome

105
Q

what are the two types of haemophilia?

A

haemophilia A and B

106
Q

haemophilia A is caused by a deficiency in what?

A

Factor VIII deficiency

107
Q

for bleeding disorders what is deemed moderate to severe?

A

<0.09 iu/ml of the factor

108
Q

for bleeding disorders what is deemed mild?

A

0.1-0.05 iu/ml of the factor

109
Q

how is severe haemophilia A managed?

A

recombinant factor VIII

110
Q

how is mild haemophilia A managed?

A

Drug DDAVP
Oral tranexamic acid

111
Q

how does the drug DDAVP help mild haemophilia A?

A

desmopressin temporarily releases factor VIII bound to endothelial cells to boost levels and clotting ability

112
Q

how does oral tranexamic acid help haemophilia A?

A

prevent fibrinolysis - clot breakdown

113
Q

what is another name for haemophilia B?

A

christmas disease

114
Q

what is haemophilia B caused by a deficiency in?

A

Factor IX deficiency

115
Q

how is severe/moderate haemophilia B managed?

A

recombinant factor IX

116
Q

how is mild/carriers haemophilia B managed?

A

recombinant factor IX

117
Q

why are the drugs DDAVP and tranexamic acid not used to treat haemophilia B?

A

No other alternatives as not bound to endothelial cells and tranexamic acid not enough

118
Q

management of severe haemophilia (<0.9iu/ml) in dental setting

A

Dental unit attached to haemophilia centre

Non risk treatments in primary care e.g. pros

Observe pt overnight after extractions and surgery

119
Q

management of mild/carriers haemophilia (0.1-0.5iu/ml)

A

Shared between public dental service and GDP
Extractions and surgery done in haemophilia unit and observed for 2-3hrs

120
Q

how should a pt with severe/moderate haemophilia be managed after extraction/surgery?

A

Observe pt overnight after extractions and surgery

121
Q

how should a pt with mild/carrier haemophilia be managed after extraction/surgery?

A

Extractions and surgery done in haemophilia unit and observed for 2-3hrs

122
Q

what should u avoid with pt with haemophilia?

A

LA blocks and lingual infiltration

123
Q

what are coagulation factor inhibitors

A

antibodies that form in response to recombinant factor VIII and IX.

124
Q

why is it important to give recombinant factor VIII and IX as infrequently as possible

A

each time they are given an antibody response occurs producing coagulation factor inhibitors. the next dose will need to be much greater to overcome these

125
Q

what is thrombophilia?

A

too much clot formed

Clot formation ability greater than clot breakdown leading to excessive stable clot formed inappropriately

126
Q

what can thrombophilia lead to?

A

pulmonary embolism

127
Q

name the two ways in which people can get thrombophilia?

A

inherited hyper coagulation

acquired hyper coagulation

128
Q

what can cause inherited hyper coagulation (thrombophilia)?

A

Protein C and S deficiency, factor V Leiden, antithrombin III deficiency

129
Q

what can cause aqcuired hyper coagulation?

A

Oral contraceptives, surgery, trauma, cancer, pregnancy, immobilisation, antiphospholipid syndrome (lupus anticoagulants)

130
Q

what is thrombocytopenia?

A

Reduced platelet numbers

131
Q

what is thrombocythaemia?

A

Increased platelet numbers but platelets are defective

132
Q

people with thrombocythaemia are given what to prevent clot formation?

A

aspirin

133
Q

describe what a qualitative bleeding disorder that affects platelets

A

Normal platelet number but abnormal function

134
Q

dental care for pt with platelet disorders

No additional precautions if platelet count is greater than ? and no functional issues

Special care is required for:
Extractions, minor oral surgery, periodontal surgery, biopsies

A

100x10*9 per litre

135
Q

what is the most common bleeding disorder that is a combined deficiency?

A

von willebrand’s disease

136
Q

what is von willebrands disease?

A

deificiency in factor VIII and reduced platelet aggregation

Defective von Willebrand’s factor on platelets interacts badly with factor VIII so poor clot activation by platelets

137
Q

what is the management for severe/moderate von willebrand’s disease (<0.09iu/ml)

A

DDAVP - release factor VIII bound to endothelial cells temporarily

138
Q

what is the management of von willebrand’s disease for mild/carriers? (0.1-0.5iu/ml)

A

Tranexamic acid - inhibit fibrinolysis

139
Q

red blood cells have antigens on their surface. these form 2 types of systems for classifying blood. what are they?

A

ABO system -> pt can be A,B O or AB

d system (rhesus) -> + or -

140
Q

name 2 indications for blood transfusion

A

blood loss
production failure

141
Q

why should blood transfusions be avoided if possible? 2

A

due to risk of transfusion reactions and transmission of infection

142
Q

what antibodies do people with group A blood have?

A

anti-B antibodies in their plasma

143
Q

what antibodies do people with group B blood have?

A

anti-A antibodies in their plasma

144
Q

what antibodies do people with group AB blood have?

A

no antibodies

145
Q

what antibodies do people with group O blood have

A

anti-A and anti-B antibodies in their plasma

146
Q

what antigens do each group have on the surface of their red blood cells?

A
B
AB
O

A

A=A
B=B
AB= A and B
0= none

147
Q

name 3 possible complications of blood transfusions

A

Red blood cell lysis - fever, jaundice, death

Fluid overload - heart failure

Transmission of infection

148
Q

define lymphoma

A

is clonal proliferation of lymphocytes arising in a lymph node or associated tissue

149
Q

how is leukaemia different from lymphoma?

A

leukaemia is a disseminated tumour of white cells which is within circulation

lymphoma is clonal proliferation of lymphocyte arising in a lymph node or associated tissue

150
Q

symptoms of lymphoma

A

Swelling/lump in the face or neck
Fever
Excessive sweating at night
Weight loss
Loss of appetite
Breathlessness
Weakness

151
Q

what are the 4 stages of lymphoma?

A
  1. Single lymph node region or site
  2. Two or more sites on one side
  3. Both sides of the diaphragm
  4. Diffuse involvement
152
Q

name 2 types of lymphoma?

A

Hodgkin’s lymphoma

Non-Hodgkin lymphoma

153
Q

clinical presentation of hodgkin’s lymphoma

A

Painless lymphadenopathy
Fever, night sweats, weight loss, itching, infection

154
Q

cause of non-hodgkin lymphoma 3

A

infection
autoimmune disease
immunosuppression

155
Q

clinical presentation of non-hodgkin lymphoma

A

Extra-nodal disease - oropharyngeal involvement

Symptoms of marrow failure

156
Q

which has a poorer prognosis, Hodgkin or non-hodgkin lymphoma?

A

non-hodgkin lymphoma

157
Q

what is a malignant myeloma?

A
  • Malignant proliferation of plasma cells
158
Q

name 3 features of multiple myeloma

A

Monoclonal paraprotein in the blood/urine

Lytic bone lesions - fracture and pain

Excess plasma cells in marrow - marrow failure

159
Q

what is the treatment of multiple myeloma? 2

A

Biologic monoclonal antibodies

Bone turnover suppression medicines e.g. bisphosphonate

160
Q

name the 4 phases of treatment for haematological malignancy

A

induction
remission
maintenance and consolidation
relapse

161
Q

what happens in the induction phase of treatment for haematological malignancy

A

high dose chemotherapy to remove disease and return to normal state

162
Q

what happens in the remission phase of treatment for haematological malignancy?

A

pt has normal bone marrow and no evidence of disease

163
Q

what happens in the maintenance and consolidation phase of treatment for haematological malignancy?

A

pt requires low level treatment to maintain at a normal state

164
Q

what happens in the relapse phase of treatment for haematological malignancy?

A

disease comes back again and the process of induction and remission will need to be repeated

165
Q

chemotherapy targets what kind of cells?

A

cells with a high turnover rate

166
Q

remember
Chemotherapy targets cells with a high turnover rate

Radiotherapy - cytotoxic effect of ionising radiation to target tissues

Monoclonal antibodies have specific cancer antigens

A
167
Q

Haemopoietic stem cell transplant

Allogenic transplant is from who?

A

a donor

168
Q

Haemopoietic stem cell transplant

Autologous transplant is from who?

A

the pt

169
Q

Haemopoietic stem cell transplant

why is it usually stem cells from the blood rather than bone marrow?

A

don’t need to remove all bone marrow

170
Q

Haemopoietic stem cell transplant is a high risk procedure.

what are the risks? 4

A

Infection
graft vs host disease
graft failure
total marrow failure

171
Q

leukaemia is what cella lineage?

A

either myeloid or lymphoid

172
Q

The earlier in the cell line it turns neoplastic the potentially more ? the malignancy

A

aggressive

173
Q

Haematological malignancy
Usually starts with a ?? which switches off a ??? or switches on an ?. This results in ?? and cancer when there is ??, loss of ? and loss of normal ?/?

A

DNA mutation
tumour supressor gene
oncogene
clonal proliferation
uncontrolled proliferation
apoptosis
functions
products

174
Q

name the types of leukaemia? 5

A

acute or chronic

Lymphocytic, lymphoblastic or myeloid describe the point in the cell lines or cell type at fault

175
Q

what is leukaemia?

A

describes a group of cancers of the bone marrow which prevent normal manufacture of the blood

176
Q

what does leukaemia result in?

A

anaemia
infection - neutropenia
bleeding - thrombocytopenia

177
Q

what is acute lymphoblastic anaemia?

A
  • Large numbers of immature lymphocytes
178
Q

acute lymphoblastic anaemia typically occurs in what type of people?

A

children

179
Q

clinical presentation of lymphoblastic anaemia 5

A

Lymphadenopathy, tissue infiltration, fever, sweats, malaise

180
Q

acute myeloid leukaemia typically occurs in what type of ppl?

A

elderly

181
Q

clinical presentation of acute myeloid leukaemia

A

Lymphadenopathy, tissue infiltration, fever, sweats, malaise

182
Q

Chronic lymphocytic leukaemia
is ? cell based?

A

B

183
Q

what is the clinical presentation of chronic lymphocytic leukaemia?

A

Mostly asymptomatic and discovered on routine blood tests

184
Q

chronic lymphocytic leukaemia has fast or slow progression?

A

slow and may not require treatment

185
Q

what is chronic myeloid leukaemia?

A

Increase in neutrophils and their precursor

186
Q

most pt with chronic myeloid leukaemia have what?

A

Philadelphia chromosome

187
Q

clinical presentation of chronic myeloid leukaemia 6

A

Fatigue, weight loss, sweating

Anaemia, bleeding, splenomegaly