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Year 5 Haematology > Medgems 27/05/24 > Flashcards

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
Q

Dabigatran

moa?
reversible agent?

A

DOAC

inhibits thrombin - factor 2a

Idarucizumab

26
Q

protein mutated in hereditary spherocytosis

A

spectrin

autosomal dominant

27
Q

large megakaryocytes on bone marrow
elevated platelet count and increased stroke risk?

A

essential thrombocytosis

28
Q

hodgkin lymphoma staging system

A

lugano classifcation

29
Q

most common type of HL?

A

nodular sclerosing

30
Q

high INR, Aptt, thrombin time, and reduced platelets is?

A

disseminated intravascular haemorrhage

31
Q

pancreatic exocrine dysfunction in children?

most common cause?

A

cystic fibrosis
shwachman diamond

32
Q

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

A

shwachman diamond

33
Q

g6pd inheritance pattern?

A

x linked recessive

34
Q

richter transformation relates to which low grade tumour turning into?

what tyrosine kinase inhibitor can be used?

A

low grade CLL > diffuse large b cell lymphoma

ibrutinib

35
Q

Venetoclax

A

BCL2 inhibitor

BCL2 inhibits apoptosis

In other words, it inhibits an inhibitor of apoptosis in leukaemic cells)

36
Q

Target cells

A

bullseye” appearance of a dark central ring surrounded by pallor

liver disease
hypospleen
iron deficient

37
Q

CML drug which inhibits fusion protein bcr-abl?

A

imatinib
tyrosine kinase inhibitor

38
Q

bone marrow failures

what is most common?

A

fanconi (most common)
diamond blackfan
shwachman-diamond
dyskeratosis congenita

39
Q

fanconi anaemia fbc abnormality?

A

pancytopenia

40
Q

dyskeratosis congenita fbc abnormality?

A

pancytopenia

41
Q

diamond blackfan fbc abnormality?

A

red cells

42
Q

schwachman-diamond?

A

neutropenia

43
Q

whihc chain is mutated in sickle cell?

A

beta globin

44
Q

thrombocytopenia and immune complex formation but is a prothrombotic condition?

A

Heparin Induced Thrombocytopenia

Heparin-PF4 blood clot formation

45
Q

direct thrombin inhibitor?

A

bivalirudin
argotroban

46
Q

HIT antibodies against?

A

heparin and platelet factor 4

47
Q

most common reaction to packed red cell rpoduct?

A

fever
febrile non haemolytic recation

48
Q

why does febrile non haemolytic transfusion reaction occur?

A

IL-1 released by white blood cells

49
Q

Cd19 and cd5 raised, painless cervical lymphadenopathy
70yr man

A

CLL

50
Q

most common lymphoma in <15

A

burkitts’
starry sky - NHL

51
Q

lymphoma >40

A

diffuse large b cell

52
Q

ferritin found in?

A

liver

53
Q

heinz bodies?

A

denatured haemoglobin

g6pd - x linked recessive

54
Q
A

Year 5 Haematology (10 decks)

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