Haematology Flashcards

1
Q

MoA of venetoclax

A

Bcl2 inhibitor (which is anti-apoptotic –> restores apoptosis)

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2
Q

A/E of Venetoclax

A

TLS

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3
Q

MoA of ibrutinib

A

Blocks Brutons TKI

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4
Q

A/E of ibrutinib

A

AF

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5
Q

Indication for venetoclax

A

CLL refractor to FCR chemotherapy

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6
Q

Treatment of MDS with 5q deletion

A

Lenalidomide

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7
Q

High risk MDS treatment strategies

A

HSCT eligible? - transplant

Not transplant eligible - azacitidine

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8
Q

MoA of idelalisib and indication

A

Pl3 kinase inhibitor

Refractory CLL

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9
Q

anti CD33?

A

GEmtuzumab

Add to CD33 +ve AML

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10
Q

What to add for FLT3 positive AML to 7+3 induction

A

Add midostaurin

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11
Q

2 main A/E of CAR-T cell

A

CRS

ICANS

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12
Q

A/E of idelalisib

A

Increased risk opportunistic infections

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13
Q

Test for Fanconi’s anaemia

A

Chromosome fragility studies

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14
Q

CAL-R mutation associated with what FBE abnormality

A

Elevated Plt
- ET, prefibrotic PMF

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15
Q

Driver mutations for myeloproliferative disorders

A

JAK-2 - most common
CAL-R
MPL

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16
Q

Highest myeloproliferative at risk of transformation to AML

A

CML (if untreated)

Otherwise PMF

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17
Q

PRV High risk

How does this affect treatment?

A

High risk = age > 60, thrombosis, or high WCC/Plt

Add hydroxyurea to phlebotomy aiming < 0.45 and aspirin

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18
Q

High risk ET

A

Age > 60, thrombosis, Plt > 1500, symptomatic splenomegaly

Add hydroxyurea to aspirin

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19
Q

Management of PRV

A

Low risk - aspirin and venesection aiming HCT < 0.45

High risk - add hydroxyurea

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20
Q

Tear drop cells

A

Myelofibrosis

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21
Q

Dry tap

A

Myelofibrosis

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22
Q

Ruxolitinib MoA

A

JAk-2 inhibitor

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23
Q

Benefits of ruxolitinib

A

Improves splenomegaly
Improves constitutional symptoms

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24
Q

A/E of ruxolitinib

A

Cytopaenias

Increased ifnection risk

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25
Q

Pitted erythrocytes

A

Hyposplenism

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26
Q

Howell Jolly Bodies

A

Hyposplenism

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27
Q

Features of hyposplenisms, should be concerned for?

A

OPSI - overwhelming post splenectomy infection

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28
Q

Functions of spleen (3)

A

White pulp - 2nd lymphoid organ
Red pulp - filtration (RBC’s, deformed blood cells, bacteria)
Haematopoiesis - thalassaemia and PMF

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29
Q

Causes of hyposplenism

A

Anything that can acuse damage to spleen
- SCT, hSCT
- Autoimmune
- Hepatic - ALD, cirrhosis
- Splenic vasculature changes

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30
Q

Features of leukaemoid reaction

A

Toxic granulation
Toxic vacuolation
Dohle bodies - small blue inclusions

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31
Q

B cell markers

A

CD19
CD20
Kappy/lambda

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32
Q

T cell markers

A

CD3
CD4
CD5
CD8

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33
Q

Stem cell marker

A

CD34

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34
Q

Inheritance vWD

A

Autosomal dominant

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35
Q

Syndrome that has acquired vWD

A

Heyde syndrome - AS and recurrent GI bleeds

36
Q

4T’s HITS

A

Thrombocytopaenia - Plt drop >50%, not < 20
Timin - D5-10
Thrombus
No other cause

37
Q

Interactions of Xa inhibitors

A

P-glycoprotien and CYP3A4
Can’t use with inhibitors - posaconazole, ketaconazole, ritonavir

Inactivated by inducers

38
Q

Interactions of dabigatran

A

Only metabolised by P-glycoprotein

39
Q

Thrombin time
- What is it
- Use

A

Measures final step - fibrinogen to fibrin

Useful for heparin effect:
- Elevated TT but normal reptilase time (insensitive to effects of heparin)

40
Q

A globin chain - chromsome

A

16

41
Q

B globin chain - chromosome

A

11

42
Q

Variants of B globins that are due to 1 aminoacid substitution

A

HbS

HbE
HbC

43
Q

Beta tetramers

A

HbH

44
Q

Gamma tetramers

A

Barts

45
Q

Erythema nodosum, hilar adenopathy, migratory polyarthralgia, fever

A

Lofgren syndrome = sarcoid

46
Q

Mutton fat keratitis

A

Opthamic sarcoid

47
Q

Lupus pernio

A

Indurated plaques with telangiectasias on central face/neck

48
Q

White cells in donor transfusion product cause?

A

Febrile non-haemolytic transfusion reaction

49
Q

Ab’s in donor transfusion reacting with white cells in recipient cause?

A

TRALI

50
Q

Mechanism to minimise febrile non-haemolytic reaction

A

Leucodepletion

51
Q

Mechanism to prevent transfusion GvHD

A

Irradiation

52
Q

Blood product with highest risk of infection/bacterial contamination

A

Platelets - stored at room temperature

53
Q

Hodgkins immunophenotype

A

CD15
CD30

54
Q

Anti-CD30

A

Brentuximab

55
Q

Complement mediate haemolysis
- other name
- type

A

PNH

Intravascular

56
Q

FEatures of intravascular haemolysis

A

Free Hb

Urine free Hb

Urine haemosiderin

57
Q

Chronic haemolysis - Rx to prevent aplastic anaemia

A

Folate

58
Q

Favourable for AML

A

Mutated NPM1
Mutated CEBPA

59
Q

Unfavourable for AML

A

FLT3-ITD mutated
Wild type NPM1

60
Q

APLS
Lab criteria

A

One or more aPL antibodies 12 weeks apart

Anticardiolipin - IgG or IgM positive 12 weeks apart

Beta 2 glycoprotein - IgG or IgM positive 12 weeks apart

Lupus anticoagulant - 12 weeks apart

61
Q

APLS clinical criteria
- Vascular

A

1 or more deep vein, arterial thrombus
- Superficial vein does not count

62
Q

APLS pregnancy morbidity criteria

A

1 or more fetal loss > 10 weeks

3 or more fetal loss < 10 weeks

Premature birth due to pre-eclampsia, eclampsia or placental insufficiency

63
Q

Smouldering myeloma criteria

A

M protein > 30
OR
>10% BM plasma cells

No end organ damage

64
Q

MGUS criteria

A

M protein < 30
BM plasma cells < 10%
No end organ damage

65
Q

Acute proximal DVT/PE management

A

3 months (for provoked/unprovoked)

Assess at 3 months chance of recurrence

66
Q

When to continue anticoagulation > 3 months for DVT/PE

A

> 2 DVT

APLS

Active cancer

67
Q

Distal (beyond popliteal) provoked DVT - length of anticoagulation

A

6 weeks

68
Q

HbS present
No HbA

2 options?

A

Normal HbA2 - Beta S and Beta S

Increased HbA2 - Beta S and Beta 0

69
Q

HbS present

HbA detected

2 options?

A

High HbA - Beta S and normal beta (trait)

High HbA2 - Beta S and Beta +

70
Q

Protamine MoA
-Heparin
- LMWH

A

Heparin - binds heparin, forms stable salt and fully nullifies drug

LWMH - incompletely reverses anti Xa activity of LMWH

71
Q

Mechanism of reduced haptoglobin in haemolytic anaemia and how cleared

A

Haptoglobin-Hb dimers clared by liver

72
Q

Protective genetic factors for Sickle cell

A

Hb F levels - prevent HbS polymerisation

Alpha thalassaemia - indirectly inhibits HbS polymerisation

73
Q

Gene high prevalence African people that is protective against Sickle cell crises

A

PKLR - pyruvate kinase

74
Q

Medication increases Hb F lvels

A

Hydroxyurea

75
Q

Medication prevents polymerisation of Hb S

A

Voxelotor

76
Q

Anti Xa assay
- Useful for?

A

LMWH monitoring

Heparin monitoring with baseline high apTT

77
Q

Anti X chromogenic assay
- Useful for?

A

Warfarin monitoring with baseline high apTT/INR

78
Q

Anaemia chronic disease vs IDA + ACD

A

ACD - Normocytic, high ferritin, low tsats

IDA and ACD - microcytic, high/normal ferritin, low tsats

79
Q

What does AT III inactivate?

A

IXa, Xa, XIa, XIIa

80
Q

what does protein C/S inactivate?

A

Factor V and VIIIa

These are needed to activate thrombin

–> having lack of protein C/S means unreguated thrombin formation

81
Q

NAPDH deficiency in RBC’s?

2nd to ?
Significance

A

G6PD deficiency (this enzyme makes NADP –> NADPH)

Lack of NADPH means more susceptible to oxidative injury

82
Q

Marrow stimulating agent used in aplastic anaemia

A

Eltrombopag

83
Q

THrombopoeitn made where?

A

Liver

84
Q

Low (but not absent) reticulocyte count

A

Folate/B12/Fe/Copper deficiency

85
Q

2nd causes of hypogammaglobulinaemia
- broad categories and causes

A

Reduce production
- drugs - steroid, rituximab
- malignancy - CLL, lymphoma, MM

Increased loss
- protein losing enteropathy
- Nephrotic syndrome
- Burns

86
Q

Mutation of JAK2 in myeloproliferative disorders - what effect?

A

Persistent active transcription and replication of cells