other Flashcards

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
what are ADP receptor antagonists
Clopidogrel and Prasugrel
26
what is a phosphodiesterase inhibitor
Dipyridamole
27
what drug is a GP2b/3a inhibitor
abciximab
28
how do you monitor unfractionated heparin
APTT
29
monitoring for LMWH
no usually needed as more predictable response
30
what can reverse heparin in severe bleeding
protamine sulpahte
31
what drug should be taken at the same time every day
warfarin
32
dabigatran MOA
direct thrombin inhibitor
33
moa of edoxaban, rivaroxaban, apixaban)
factor X inhibitors
34
haematopoiesis is the term used
for the production of blood cells
35
adult where is haemopoiesis restricted to a
axial skeleton
36
granulocytes include what
neutrophils, eosinophils and basophils
37
what cell are bi lobed and usually have bright red/ orange granules
eosinophils
38
what do basophils look like
large deep purple granules obscuring nucleus
39
what cell has a large single nucleus, faintly staining granules and are often vacuolated
monocyte
40
small with condensed nucleus and rim of cytoplasm, when activated appear large with plentiful blue cytoplasm extending round neighbouring red cells
lymphocyte
41
what shows lineage of progeny
bioassays
42
how do red cells have a limited lifespan
there is no nucleus so they can't divide or replace damaged proteins
43
adult haemoglobin is
2 alpha and 2 beta chains
44
red cell production is regulated by what and is produced in hypoxia
erythropoietin
45
red cell destruction normally occurs where
Spleen (and liver)
46
the heme group is broken down into what
iron and bilirubin
47
NADPH replenishes what in the hexose monophosphate shunt
glutathione
48
in the hexose monophosphate shunt what is the rate limiting enzyme
Glucose -6 - phosphate dehydrogenase
49
most co2 is transported with
bicarbonate
50
what is increased in chronic anaemia
2-3 DPG
51
where does iron absorption mainly happen
duodenum
52
what decreases iron absorption
tea, calcium, phylates
53
what increases iron absorption
ascoribic acid and alcohol
54
what is the major negative regulator of iron uptake
hepcidin
55
what levels decrease when iron deficient
hepcidin
56
molecule that is spherical and stores iron
ferritin
57
what measures functional iron
haemoglobin conc
58
what also acts as an acute phase protein so goes up with infection, malignancy, liver injury etc
serum ferritin
59
what is secondary haemostasis
formation of fibrin clot
60
von willebrand factor deficiency has what inheritance
autosomal dominant
61
drugs that can reduce platelet function
aspirin or NSAIDs
62
the only really screening test for primary haemostasis
platelet count
63
what converts prothrombin to thrombin
V/Xa
64
What converts fibrinogen to fibrin
thrombin
65
what is a single clotting factor deficiency
haemophilia
66
what has an isolated prolonged APTT
haemophilia
67
examples of what has multiple clotting factor deficiences
DIC, liver failure (as all coagulation factors are synthesises in the liver)
68
prothrombin time relates to what pathway
extrinsic
69
what is used as a measure of overall clotting
prothrombin time
70
APTT measures what factors
8,9,11 and 12
71
most common causes of increased APTT are
haemophilia and possibly von willebrands disease
72
most common reasons for both prothrombin and APTT to be increase
due to multiple clotting factor deficiencies eg liver diease, anticoagulants
73
what breaks down fibrin
plasmin
74
anti- thrombin, protein C and S are
anticoagulant defences
75
what cell only lives for 7-8hrs
neutrophils
76
what are nucleated precursor cells
blasts
77
what are immediate red cell precursor
reticulocytes
78
what means you have more immature red blood cells than what considered normal
polychromasia
79
what is a myelocyte
nucleated precursor between neutrophils and myeloblasts
80
what is used for bone marrow biopsy in a young child
tibia
81
what marrow increases with age
yellow
82
are lymph nodes primary or secondary lymphoid tissues
secondary
83
where does B cell maturation occur
bone marrow
84
where does T cell maturation occur
thymus
85
what things stimulate a predominant T cell response
viral infections, drugs
86
what cell response predominates in autoimmune conditions and infections
B
87
what is troiseries sign
manifestation of metastasis from an abdominal malignancy
88
what is a sentinel lymph node
first lymph node to which cancer cells are more likely to spread
89
where will metastatic cells be identified first
subscapular sinus
90
where is the spleen located
upper left quadrant of the abdomen
91
there can be lack of differentiation or maturation in
malignant haemopoiesis
92
in what there is a proliferation of abnormal progenitors with a block in differentiation/maturation
acute leukaemia. in acute there is a block and in chronic there is no differentiation/ maturation
93
is normal haeompoiesis mono or poly clonal
polyclonal
94
myeloma is malignancy of what cell
plasma
95
CLL affect more what
mature B lymphocytes
96
leukaemia affects blood and lymphoma affects the lymph node. but what is an exception
CLL - can involve both
97
what leukemias are usually more aggressive
acute
98
immunoglobulin are produces by what cells
B and plasma
99
where do B cells encounter the antigen
follicle germinal centre
100
what is a monoclonal immunoglobulin
paraprotein
101
what allows for identification of an abnormal protein band
serum electrophoresis
102
what allows to classify the abnormal protein band
serum immunofixation
103
bence jones protein has immunoglobulin ... chains
light
104