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1
Q

what are insoluble protein fibrils

A

amyloid

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2
Q

most common presenting symptom of amyloidosis

A

weakness and dyspnoea

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3
Q

what amyloidosis refers to light chain and what one refers to inflammatory

A

Light - AL
inflammatory - AA

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4
Q

commonest reason doe hereditary haemochromatosis

A

mutation in HFE gene

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5
Q

mx for hereditary haemochromatosis

A

weekly venesection

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6
Q

mx for secondary iron overload

A

iron chelating agents eg desferrioxamine

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7
Q

increased destruction cause of pancytopenia

A

hypersplenism

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8
Q

causes of hypersplenism

A

portal hypertension, systemic disease eg RA, haematological disease eg splenic lymphoma

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9
Q

what pancytopenia has hypocellularity

A

aplastic anaemia

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10
Q

minimum criteria for blood transfusion donor

A

Hb 125 for women and 135 for men and weight 50kg

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11
Q

ABO gene is on chromosome

A

9

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12
Q

O blood can only receive what blood

A

0 blood

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13
Q

what is antisera

A

using reagents with known antibody specificity to identify antigens present on red cell

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14
Q

what does agglutination indicate

A

presence of an antibody

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15
Q

when do you infuse FFP

A
  • Treatment of bleeding in patient with coagulopathy (PT ratio >1.5)
  • Prior to surgery or procedure in patient with coagulopathy (PT ratio >1.5)
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16
Q

what is febrile non haemolytic transfusion reaction

A

presents with fever, rigours/chills but patient otherwise well

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17
Q

mx of febrile non haemolytic transfusion reaction

A

slow the transfusion and give paracetamol

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18
Q

which protein modulates transferrin uptake (iron overload)

A

HFE

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19
Q

what can lower iron levels in iron overload

A

desferrioaxamine

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20
Q

patients post splenectomy have what

A

Howell-jolly bodies and Pappenheimer bodies

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21
Q

highest risk of infection after splenectomy is when and with what bacteria

A

2 years after and from pneumococcal

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22
Q

lifelong antibiotics prophylaxis post splenectomy is what antibiotic

A

Phenoxymethylpenicllin also know as penicillin V

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23
Q

COX is necessary to produce what

A

thromboxane 2

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24
Q

what should be stopped 7 days prior to elective operations

A

anti platelet agents

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25
Q

what are ADP receptor antagonists

A

Clopidogrel and Prasugrel

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26
Q

what is a phosphodiesterase inhibitor

A

Dipyridamole

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27
Q

what drug is a GP2b/3a inhibitor

A

abciximab

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28
Q

how do you monitor unfractionated heparin

A

APTT

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29
Q

monitoring for LMWH

A

no usually needed as more predictable response

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30
Q

what can reverse heparin in severe bleeding

A

protamine sulpahte

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31
Q

what drug should be taken at the same time every day

A

warfarin

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32
Q

dabigatran MOA

A

direct thrombin inhibitor

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33
Q

moa of edoxaban, rivaroxaban, apixaban)

A

factor X inhibitors

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34
Q

haematopoiesis is the term used

A

for the production of blood cells

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35
Q

adult where is haemopoiesis restricted to a

A

axial skeleton

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36
Q

granulocytes include what

A

neutrophils, eosinophils and basophils

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37
Q

what cell are bi lobed and usually have bright red/ orange granules

A

eosinophils

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38
Q

what do basophils look like

A

large deep purple granules obscuring nucleus

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39
Q

what cell has a large single nucleus, faintly staining granules and are often vacuolated

A

monocyte

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40
Q

small with condensed nucleus and rim of cytoplasm, when activated appear large with plentiful blue cytoplasm extending round neighbouring red cells

A

lymphocyte

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41
Q

what shows lineage of progeny

A

bioassays

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42
Q

how do red cells have a limited lifespan

A

there is no nucleus so they can’t divide or replace damaged proteins

43
Q

adult haemoglobin is

A

2 alpha and 2 beta chains

44
Q

red cell production is regulated by what and is produced in hypoxia

A

erythropoietin

45
Q

red cell destruction normally occurs where

A

Spleen (and liver)

46
Q

the heme group is broken down into what

A

iron and bilirubin

47
Q

NADPH replenishes what in the hexose monophosphate shunt

A

glutathione

48
Q

in the hexose monophosphate shunt what is the rate limiting enzyme

A

Glucose -6 - phosphate dehydrogenase

49
Q

most co2 is transported with

A

bicarbonate

50
Q

what is increased in chronic anaemia

A

2-3 DPG

51
Q

where does iron absorption mainly happen

A

duodenum

52
Q

what decreases iron absorption

A

tea, calcium, phylates

53
Q

what increases iron absorption

A

ascoribic acid and alcohol

54
Q

what is the major negative regulator of iron uptake

A

hepcidin

55
Q

what levels decrease when iron deficient

A

hepcidin

56
Q

molecule that is spherical and stores iron

A

ferritin

57
Q

what measures functional iron

A

haemoglobin conc

58
Q

what also acts as an acute phase protein so goes up with infection, malignancy, liver injury etc

A

serum ferritin

59
Q

what is secondary haemostasis

A

formation of fibrin clot

60
Q

von willebrand factor deficiency has what inheritance

A

autosomal dominant

61
Q

drugs that can reduce platelet function

A

aspirin or NSAIDs

62
Q

the only really screening test for primary haemostasis

A

platelet count

63
Q

what converts prothrombin to thrombin

A

V/Xa

64
Q

What converts fibrinogen to fibrin

A

thrombin

65
Q

what is a single clotting factor deficiency

A

haemophilia

66
Q

what has an isolated prolonged APTT

A

haemophilia

67
Q

examples of what has multiple clotting factor deficiences

A

DIC, liver failure (as all coagulation factors are synthesises in the liver)

68
Q

prothrombin time relates to what pathway

A

extrinsic

69
Q

what is used as a measure of overall clotting

A

prothrombin time

70
Q

APTT measures what factors

A

8,9,11 and 12

71
Q

most common causes of increased APTT are

A

haemophilia and possibly von willebrands disease

72
Q

most common reasons for both prothrombin and APTT to be increase

A

due to multiple clotting factor deficiencies eg liver diease, anticoagulants

73
Q

what breaks down fibrin

A

plasmin

74
Q

anti- thrombin, protein C and S are

A

anticoagulant defences

75
Q

what cell only lives for 7-8hrs

A

neutrophils

76
Q

what are nucleated precursor cells

A

blasts

77
Q

what are immediate red cell precursor

A

reticulocytes

78
Q

what means you have more immature red blood cells than what considered normal

A

polychromasia

79
Q

what is a myelocyte

A

nucleated precursor between neutrophils and myeloblasts

80
Q

what is used for bone marrow biopsy in a young child

A

tibia

81
Q

what marrow increases with age

A

yellow

82
Q

are lymph nodes primary or secondary lymphoid tissues

A

secondary

83
Q

where does B cell maturation occur

A

bone marrow

84
Q

where does T cell maturation occur

A

thymus

85
Q

what things stimulate a predominant T cell response

A

viral infections, drugs

86
Q

what cell response predominates in autoimmune conditions and infections

A

B

87
Q

what is troiseries sign

A

manifestation of metastasis from an abdominal malignancy

88
Q

what is a sentinel lymph node

A

first lymph node to which cancer cells are more likely to spread

89
Q

where will metastatic cells be identified first

A

subscapular sinus

90
Q

where is the spleen located

A

upper left quadrant of the abdomen

91
Q

there can be lack of differentiation or maturation in

A

malignant haemopoiesis

92
Q

in what there is a proliferation of abnormal progenitors with a block in differentiation/maturation

A

acute leukaemia. in acute there is a block and in chronic there is no differentiation/ maturation

93
Q

is normal haeompoiesis mono or poly clonal

A

polyclonal

94
Q

myeloma is malignancy of what cell

A

plasma

95
Q

CLL affect more what

A

mature B lymphocytes

96
Q

leukaemia affects blood and lymphoma affects the lymph node. but what is an exception

A

CLL - can involve both

97
Q

what leukemias are usually more aggressive

A

acute

98
Q

immunoglobulin are produces by what cells

A

B and plasma

99
Q

where do B cells encounter the antigen

A

follicle germinal centre

100
Q

what is a monoclonal immunoglobulin

A

paraprotein

101
Q

what allows for identification of an abnormal protein band

A

serum electrophoresis

102
Q

what allows to classify the abnormal protein band

A

serum immunofixation

103
Q

bence jones protein has immunoglobulin … chains

A

light

104
Q
A

My haem (8 decks)

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