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Haematology Flashcards

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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
Pitted erythrocytes
Hyposplenism
26
Howell Jolly Bodies
Hyposplenism
27
Features of hyposplenisms, should be concerned for?
OPSI - overwhelming post splenectomy infection
28
Functions of spleen (3)
White pulp - 2nd lymphoid organ Red pulp - filtration (RBC's, deformed blood cells, bacteria) Haematopoiesis - thalassaemia and PMF
29
Causes of hyposplenism
Anything that can acuse damage to spleen - SCT, hSCT - Autoimmune - Hepatic - ALD, cirrhosis - Splenic vasculature changes
30
Features of leukaemoid reaction
Toxic granulation Toxic vacuolation Dohle bodies - small blue inclusions
31
B cell markers
CD19 CD20 Kappy/lambda
32
T cell markers
CD3 CD4 CD5 CD8
33
Stem cell marker
CD34
34
Inheritance vWD
Autosomal dominant
35
Syndrome that has acquired vWD
Heyde syndrome - AS and recurrent GI bleeds
36
4T's HITS
Thrombocytopaenia - Plt drop >50%, not < 20 Timin - D5-10 Thrombus No other cause
37
Interactions of Xa inhibitors
P-glycoprotien and CYP3A4 Can't use with inhibitors - posaconazole, ketaconazole, ritonavir Inactivated by inducers
38
Interactions of dabigatran
Only metabolised by P-glycoprotein
39
Thrombin time - What is it - Use
Measures final step - fibrinogen to fibrin Useful for heparin effect: - Elevated TT but normal reptilase time (insensitive to effects of heparin)
40
A globin chain - chromsome
16
41
B globin chain - chromosome
11
42
Variants of B globins that are due to 1 aminoacid substitution
HbS HbE HbC
43
Beta tetramers
HbH
44
Gamma tetramers
Barts
45
Erythema nodosum, hilar adenopathy, migratory polyarthralgia, fever
Lofgren syndrome = sarcoid
46
Mutton fat keratitis
Opthamic sarcoid
47
Lupus pernio
Indurated plaques with telangiectasias on central face/neck
48
White cells in donor transfusion product cause?
Febrile non-haemolytic transfusion reaction
49
Ab's in donor transfusion reacting with white cells in recipient cause?
TRALI
50
Mechanism to minimise febrile non-haemolytic reaction
Leucodepletion
51
Mechanism to prevent transfusion GvHD
Irradiation
52
Blood product with highest risk of infection/bacterial contamination
Platelets - stored at room temperature
53
Hodgkins immunophenotype
CD15 CD30
54
Anti-CD30
Brentuximab
55
Complement mediate haemolysis - other name - type
PNH Intravascular
56
FEatures of intravascular haemolysis
Free Hb Urine free Hb Urine haemosiderin
57
Chronic haemolysis - Rx to prevent aplastic anaemia
Folate
58
Favourable for AML
Mutated NPM1 Mutated CEBPA
59
Unfavourable for AML
FLT3-ITD mutated Wild type NPM1
60
APLS Lab criteria
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
APLS clinical criteria - Vascular
1 or more deep vein, arterial thrombus - Superficial vein does not count
62
APLS pregnancy morbidity criteria
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
Smouldering myeloma criteria
M protein > 30 OR >10% BM plasma cells No end organ damage
64
MGUS criteria
M protein < 30 BM plasma cells < 10% No end organ damage
65
Acute proximal DVT/PE management
3 months (for provoked/unprovoked) Assess at 3 months chance of recurrence
66
When to continue anticoagulation > 3 months for DVT/PE
>2 DVT APLS Active cancer
67
Distal (beyond popliteal) provoked DVT - length of anticoagulation
6 weeks
68
HbS present No HbA 2 options?
Normal HbA2 - Beta S and Beta S Increased HbA2 - Beta S and Beta 0
69
HbS present HbA detected 2 options?
High HbA - Beta S and normal beta (trait) High HbA2 - Beta S and Beta +
70
Protamine MoA -Heparin - LMWH
Heparin - binds heparin, forms stable salt and fully nullifies drug LWMH - incompletely reverses anti Xa activity of LMWH
71
Mechanism of reduced haptoglobin in haemolytic anaemia and how cleared
Haptoglobin-Hb dimers clared by liver
72
Protective genetic factors for Sickle cell
Hb F levels - prevent HbS polymerisation Alpha thalassaemia - indirectly inhibits HbS polymerisation
73
Gene high prevalence African people that is protective against Sickle cell crises
PKLR - pyruvate kinase
74
Medication increases Hb F lvels
Hydroxyurea
75
Medication prevents polymerisation of Hb S
Voxelotor
76
Anti Xa assay - Useful for?
LMWH monitoring Heparin monitoring with baseline high apTT
77
Anti X chromogenic assay - Useful for?
Warfarin monitoring with baseline high apTT/INR
78
Anaemia chronic disease vs IDA + ACD
ACD - Normocytic, high ferritin, low tsats IDA and ACD - microcytic, high/normal ferritin, low tsats
79
What does AT III inactivate?
IXa, Xa, XIa, XIIa
80
what does protein C/S inactivate?
Factor V and VIIIa These are needed to activate thrombin --> having lack of protein C/S means unreguated thrombin formation
81
NAPDH deficiency in RBC's? 2nd to ? Significance
G6PD deficiency (this enzyme makes NADP --> NADPH) Lack of NADPH means more susceptible to oxidative injury
82
Marrow stimulating agent used in aplastic anaemia
Eltrombopag
83
THrombopoeitn made where?
Liver
84
Low (but not absent) reticulocyte count
Folate/B12/Fe/Copper deficiency
85
2nd causes of hypogammaglobulinaemia - broad categories and causes
Reduce production - drugs - steroid, rituximab - malignancy - CLL, lymphoma, MM Increased loss - protein losing enteropathy - Nephrotic syndrome - Burns
86
Mutation of JAK2 in myeloproliferative disorders - what effect?
Persistent active transcription and replication of cells

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