haem Flashcards

1
Q

chronic myeloid leukaemia is caused by what mutation?

A

(T9;22) philadelphia

BCR-ABL1 re-arrangement

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2
Q

what types of leukaemia’s have blocked differentiation/maturation

A

Acute leukaemia’s

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3
Q

define acute leukaemia

A

rapidly progressive clonal malignancy of the marrow/blood with maturation defect(S)

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4
Q

outline ALL

A
  • Malignant disease of primitive lymphoid cells
  • most common childhood cancer
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5
Q

ALL can involve areas outside bone marrow true/false

A

true

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6
Q

some patients with AML can present with what?

A

Coagulation defect (DIC)

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7
Q

what condition can Auer rods be seen

A

AML only

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8
Q

what investigation is needed for a definitive diagnosis of AML/ALL

A

immunophenotyping

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9
Q

how long can ALL treatment last

A

2-3 years (varying intensity)

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10
Q

outline AML treatment

A

intensive chemo (3-4 cycles)

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11
Q

what type of bacteria can cause fulminant life-threatening sespsis in neutropenic patients?

A

Gram negative bacteria

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12
Q

if there is a prolonged neutropenia and persisting fever unresponsive to anti- bacterial agents give ________

A

Anti- fungal

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13
Q

define CLL

A

A clonal (malignant) lymphoproliferative disorder of the mature B lymphocyte compartment

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14
Q

‘smudge/Smear Cell’

A

CLL

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15
Q

what is the difference between leukaemia and lymphoma

A

leukaemia - bone marrow and blood
lymphoma - lymph nodes

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16
Q

differential diagnosis for lymphadenopathy

A

‘reactive’
bacteria infection (regional)
viral infection (generalised)
metastatic malignancy
lymphoma

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17
Q

what IGM are associated with warm and cold AHA

A

COLD - IgM
WARM - IgG

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18
Q

what is the first line prophylactic antibiotic following splenectomy

A

Phenoxymethylpenicillin

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19
Q

smudge cells

A

CLL

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20
Q

reed-steernberg cells

A

hodgkins lymphoma

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21
Q

diagnosis of sickle cell anaemia involves

A

haemoglobin electrophoresis

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22
Q

teardrop poikilocytes

A

myelofibrosis

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23
Q

Which factors do protein C and S inhibit in the clotting cascade?

A

1985
10a
9
8
5

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24
Q

Normocytic anaemia with normal ferritin, in the presence of an inflammatory or malignant condition + treatment

A

anaemia of chronic disease
supportive treatment+ treat underlying cause

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25
Q

prolonged PT, while aPTT and TT are normal

A

factor VII deficiency

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26
Q

the first type of white blood cell to reach areas of acute inflammation

A

neutrophils

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27
Q

CLL can transform into non-hodgins lymphoma what is this called (B-Cells)

A

richters transformation

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28
Q

first line treatment for immune thrombocytopenic purpura

A

cortico steroids - increase platelet production and decrease platelet destruction

29
Q

what is a common cause of erythema infectiosum ‘fifth disease’

A

parvovirus B19

30
Q

dry tap on bone marrow aspiration

A

myelofibrosis

31
Q

iron results due to anaemia of chronic disease

A

Low serum iron, high ferritin and low transferrin saturation

32
Q

schistocytes

A

intravascular haemolysis

33
Q

what virus is associated with burkitts lymphoma

A

EBV

33
Q

Raised red cell mass, low serum erythropoetin, JAK2 mutation present

A

PCV

33
Q

howell-jolly bodies

A

hyposplenism

34
Q

typical history for g6pd deficiency

A
  • Neonatal jaundice
  • Infection/drugs precipitate haemolysis
  • Gallstones
34
Q

give the typical gender + ethnicity for g6pd deficiency

A

male (x-linked recessive) african + mediterranean descent

34
Q

gender and ethnicity of hereditery spherocytosis

A

Male + female (autosomal dominant)
northern European descent

34
Q

typical history for hereditary spherocytosis

A
  • Neonatal jaundice
    *aplastic crisis precipitated by parvovirus infection
  • Gallstones
  • Splenomegaly is common
  • MCHC elevated
35
Q

blood film and diagnostic test for g6pd deficiency

A

heinz bodies, bite and blister cells
Measure enzyme activity of G6PD

36
Q

blood film and diagnostic test for hereditary spherocytosis

A

spherocytes

EMA binding

37
Q

give long term treatment for hereditery spherocytosis

A

folate replacement
splenectomy

38
Q

pathophysiology of g6pd deficiency

A

↓ G6PD → ↓ reduced NADPH → ↓ reduced glutathione → increased red cell susceptibility to oxidative stress

39
Q

outline extrinsic pathway

A

tissue factor/factor VII - monitered by PTT

40
Q

outline intinsic pathway

A

XII/XI => VIII/IX – monitored by APTT

41
Q

sickle cell inheritance pattern

A

autosomal recessive

42
Q

starry sky appearance

A

burkitts lymphoma (non-hodgkins)

43
Q

tumour lysis investigationresults

A

ECG, U&E (potassium and phosphate, uric acid are typically raised), calcium (low),

44
Q

Normal platelets; prolonged bleeding time; prolonged APTT, normal PT and TT

A

von willebrand

45
Q

first-line clinical treatment for tumour lysis syndrome

A

Rasburicase - recombinant urate oxidase, meaning it metabolises uric acid to allantoin

46
Q

which pathogen can cause a sickle-cell crises

A

strep. pneumoniae

47
Q

burkitts lymphoma mutation

A

Translocation between chromosomes 8 and 14 is associated with Burkitt’s lymphoma

48
Q

CLL treatment and MOA

A

rimatunib - tyrosine kinase inhibitor

49
Q

mylodyplastic syndrome can turn into

A

aml

50
Q

what can polycythaemia vera turn into

A

AML or myelofibrosis

51
Q

prosthetic heart valves may result in

A

haemolytic anaemia

52
Q

Isolated rise in GGT in the context of a macrocytic anaemia suggests

A

alcohol excess

53
Q

thalasseamia diagnosis

A

HPLC

54
Q

burr cells

A

uraemia

55
Q

Schistocytes or helmet cells

A

microangiopathic haemolytic anaemia

56
Q

Rouleaux formation

A

multiple myeloma

57
Q

Drugs causing folate deficiency

A

phenytoin, methotrexate, trimethoprim

58
Q

treatment for warm haemolytic anaemia

A

prednisolone, splenectomy

59
Q

Widespread petechiae in child 2-week post infection

A

immune thrombocytopaenia

60
Q

RAT FN (renal failure, anaemia, thrombocytopaenia, fever, neuro dysfunction)

A

TTP

61
Q

treatment for TTP

A

plasma and prednisolone

62
Q

increased basophils

A

CML

63
Q

Most common Hodgkin’s lymphoma

A

nodular sclerosing