Medgems 27/05/24 Flashcards

1
Q

PT has beta thalassaemia
pre transfusion bloods show a raised WCC and low haemoglobin

clinically well
apyrexial

what blood test do you need?
why?

A

reticulocyte count

ribosomes in immature RBC stain darkly as they are basophilic

so can appear as WCC

need to differentiate to know what is happening especially as a she has beta thalassaemia
reticulocytes can be misread as neutrophils

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2
Q

what is tumour lysis syndrome?

what complications can arise?

A

rapid destruction of tumour cells = intracellular release

renal failure
arrythmia
death

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3
Q

what does the cairo bishop score ?

A

potassium >6
phosphate >1.125
calcium <1.75
urate >475umol/l

TLS

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4
Q

how to manage TLY

A

IV allopurinol
rasburicase

pre treatment

monitoring is mainstay

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5
Q

Man presents to his out of hours GP with the following symptoms

nausea with vomiting
lack of appetite and palpitations

these came on in the last hour
he had just got home from his chemo appointment

A

tumour lysis syndrome

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6
Q

what is low in vWF disease?

A

APTT prolonged
low factor 8
and prolonged bleeding time

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7
Q

what is the link between vWF and factor 8

A

factor 8 is bound to vWF so in vWF disease factor 8 is rapidly degraded

affects aptt

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8
Q

why does haemophilia not prolong bleeding time?

A

it is a clotting disorder

bleeding is determined by platelets and vWF which are normal in haemophila

A- factor8
B-9

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9
Q

can a 20 year old girl have haemophilia A?

A

haemophilia is x-linked
so must have a copy from both dad and mum

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10
Q

t(8;14)

A

burkitt’s
cmyc

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11
Q

BCR-ABL fusion gene is which transloctaion?

A

9;22

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12
Q

t(14;18)

A

follicular lymphoma
BCL-2

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13
Q

what is relevance of myc gene?

A

put’s it next to a immunoglobulin making gene

myc causes division

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14
Q

sheets of large lymphocytes

A

diffuse

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15
Q

macrophages clearly contain apoptotic lymphocyte would be?

what is this appearance described as on microscopy?

A

starry sky

phagocytosed lymphocytes
c-MYC gene translocation 8;14

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16
Q

hereditary elliptocytosis

A

autosomal dominant

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17
Q

unexplained IDA should be investigated how?

A

OGD
colonoscopy
urine dip and Ix for coeliac

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18
Q

IDA in developing world?

A

hookworm

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19
Q

fibrin degredation product blood test?

A

D dimer

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20
Q

haptoglobin measures?

A

normally binds free Hb released from RBC during haemolysis

to tag it to be cleared by reticuloendothelial system

low haptoglobin means haemolysis is happening faster than haptoglobin can bind to free haemoglobin

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21
Q

pencil cell other names?

A

cigar
helmet

can suggest schistocyte

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22
Q

genetic condition that affects the synthesis of haem molecule; haemoglobin, peroxidase , p450

A

porphyria

cytochrome p450 enzymes

23
Q

thrombocytosis in a smoker is a reason for urgent 2ww referral for suspected lung cancer?

T/F

A

true

DVT in upper limb consider extrinisic compression of upper limb venous return

also think malignancy if upper limb DVT ; d dimer and platelet high

as hypercoagulable blood in malignancy

24
Q

Rivaroxaban is a ?

reversal agent?

A

DOAC

andexanet alfa

25
Dabigatran moa? reversible agent?
DOAC inhibits thrombin - factor 2a Idarucizumab
26
protein mutated in hereditary spherocytosis
spectrin autosomal dominant
27
large megakaryocytes on bone marrow elevated platelet count and increased stroke risk?
essential thrombocytosis
28
hodgkin lymphoma staging system
lugano classifcation
29
most common type of HL?
nodular sclerosing
30
high INR, Aptt, thrombin time, and reduced platelets is?
disseminated intravascular haemorrhage
31
pancreatic exocrine dysfunction in children? most common cause?
cystic fibrosis shwachman diamond
32
pancreatic exocrine dysfunction neutropenia very short stature, with most patients being less than the fifth percentile for height and a preponderance of skeletal issues autosomal recessive
shwachman diamond
33
g6pd inheritance pattern?
x linked recessive
34
richter transformation relates to which low grade tumour turning into? what tyrosine kinase inhibitor can be used?
low grade CLL > diffuse large b cell lymphoma ibrutinib
35
Venetoclax
BCL2 inhibitor BCL2 inhibits apoptosis In other words, it inhibits an inhibitor of apoptosis in leukaemic cells)
36
Target cells
bullseye" appearance of a dark central ring surrounded by pallor liver disease hypospleen iron deficient
37
CML drug which inhibits fusion protein bcr-abl?
imatinib tyrosine kinase inhibitor
38
bone marrow failures what is most common?
fanconi (most common) diamond blackfan shwachman-diamond dyskeratosis congenita
39
fanconi anaemia fbc abnormality?
pancytopenia
40
dyskeratosis congenita fbc abnormality?
pancytopenia
41
diamond blackfan fbc abnormality?
red cells
42
schwachman-diamond?
neutropenia
43
whihc chain is mutated in sickle cell?
beta globin
44
thrombocytopenia and immune complex formation but is a prothrombotic condition?
Heparin Induced Thrombocytopenia Heparin-PF4 blood clot formation
45
direct thrombin inhibitor?
bivalirudin argotroban
46
HIT antibodies against?
heparin and platelet factor 4
47
most common reaction to packed red cell rpoduct?
fever febrile non haemolytic recation
48
why does febrile non haemolytic transfusion reaction occur?
IL-1 released by white blood cells
49
Cd19 and cd5 raised, painless cervical lymphadenopathy 70yr man
CLL
50
most common lymphoma in <15
burkitts' starry sky - NHL
51
lymphoma >40
diffuse large b cell
52
ferritin found in?
liver
53
heinz bodies?
denatured haemoglobin g6pd - x linked recessive
54