Haematology

Question 1

Lab criteria APLS

Answer 1

1. Lupus anticoagulant
   > Prolonged APTT that fails to correct with mixing with prolonged reptiliase time
2. Anticardiolipin antibody >99th percentile
3. Anti-B2 glycoprotein -1 antibody >99th percentile

Must be present on 2 occasions 12 weeks apart

Question 2

Clinical criteria APLS

Answer 2

One or more arterial, venous thrombosis

One of more unexplained fetal deaths over 10 weeks gestation

One or more Premature birth before 34 weeks

Three or more unexplained miscarriages before 10 weeks

Question 3

Anticoagulation for Antiphospholipid Ab and 1 foetal loss at ten weeks

Answer 3

Low dose aspirin/prophylactic LMWH during pregnancy

Post partum prophylaxis

Question 4

Antiphospholipid Ab and no thrombosis or pregnancy complications

Answer 4

Post partum prophylaxis for 6/52

Question 5

Pregnancy anticoagulation for Lab criteria + Clinical pregnancy criteria of 3 or more pregnancy losses

Answer 5

Antepartum prophylactic clexane + aspirin

Question 6

Dabigatran benefit over warfarin in AF

Answer 6

Less ICH
Increased GI bleeding
Small increase in myocardial infarction

Question 7

Dabigatran reversal agent

Answer 7

Idarucizumab 5g

Question 8

Factors in Prothrombin X

Answer 8

IX, X, II

Question 9

Tranexamic acid mechanism

Answer 9

inhibits the activation of plasminogen via binding at several distinct sites, the latter of which is a lysin residue involved in its binding to fibrin

Question 10

FXa inhibitor antidote

Answer 10

Andexanet alpha

Antedote for apixaban, rivaroxaban

Question 11

Factor 13

Answer 11

Coagulation factor XIII (FXIII) is converted by thrombin and Ca(2+) into an active transglutaminase (FXIIIa) in the final phase of coagulation cascade. Its main function is the mechanical stabilization of fibrin clot and its protection from fibrinolysis by cross-linking of fibrin chains and α(2)-plasmin inhibitor to fibrin

Question 12

Haemophilia C

Answer 12

Factor 11 deficiency

Question 13

FVIII/IX deficiency severity

Answer 13

<1 severe- spontaneous
1-5% mod - surgery/minor trauma
>5 mild - major trauma

Question 14

Cryoprecipitate contents

Answer 14

Factor VIII, fibrinogen, Factor XIII, vWF and fibronectin from the FFP

Only used to replace fibrinogen nowdays

Question 15

Biostate contents

Answer 15

FVIII + vWF

Question 16

Factor IX replacements

Answer 16

MonoFIX - plasma derived

Benefix - recombinant

Question 17

Desmopressin/ DDAVP (ADH)

Answer 17

Increases in FVIII and vWF

Treatment in mild T1 vWFD

Question 18

Treatment of acquired inhibitors

Answer 18

Novoseven

Question 19

Bernard-Soulier syndrome

Answer 19

Abnormality of platelet glycoprotein 1b-IX-V, a receptor for vWF

Question 20

Glanzmann thrombasthenia

Answer 20

Defect in glycoprotein IIb/IIIa complex

Question 21

Peak timing of thrombocytopaenia in gestational thrombocytopaenia

Answer 21

2 and 3rd trimester

Question 22

Mechanism and treatment of idiopathic TTP

Answer 22

Autoimmune ADAMTS13 deficiency

Treated with PLEX

Question 23

Treatent of HITTS

Answer 23

Fondaparinux

Bivalirudin - renal impaired

Question 24

action of hepcidin in Anaemia CD

Answer 24

Increased hepcidin
Blocks ferroportin
Unable to absorb iron

Question 25

HbH

Answer 25

Beta tetramers

Question 26

Hb Barts

Answer 26

Gamma tetramers

Question 27

HbA2

Answer 27

Alpha x2 Delta x 2

Question 28

Intravascular haemolytic

Answer 28

Urine haemosiderin

Question 29

Emicizumab

Answer 29

Treatment for haemophilia A | Bridges factor 9 and 10 in similar mechanism to factor 8

Question 30

Smudge/smear cells

Answer 30

CLL

Question 31

Lab/imaging reasons to treat MM

Answer 31

60% plasma cells Serum free light chains >100 reater than one focal lesion on MRI

Question 32

APML mutation

Answer 32

t(15;17)

Question 33

APML RX

Answer 33

ATRA | Arsenic

Question 34

Auer rods

Answer 34

AML

Question 35

Mode of inheritance vWD

Answer 35

Autosomal dominant

Question 36

CML cytogenetic abnormality

Answer 36

t(9;22)

Question 37

Antibody involved in haemolytic transfuion reactions

Answer 37

IgM

Question 38

Follicular lymphoma cytogenetic abnormality

Answer 38

t(14;18)

Question 39

Vincristine mechanism of action

Answer 39

Metaphase is the phase of the cell cycle when the chromosomes align at the middle of the cell and then begin to separate. Vincristine disrupts this process by binding to the tubulin protein, preventing the formation of microtubules and preventing the initiation of chromosome separation. The cell will then undergo apoptosis.

Question 40

The herpes virus Epstein-Barr establishes latent infection in which immune cell

Answer 40

B-Cells

Question 41

Pathogen associated with the development of adult T-cell leukaemia

Answer 41

HTLV-1

Question 42

CD classes associated with Reed-steinberg cells

Answer 42

CD-15 CD-30

Question 43

Marker that identifies peripheral stem cells

Answer 43

CD34

Question 44

Best method for reducing transfusion related GVHD

Answer 44

Irradiation

Question 45

Benefit of leukoreduction

Answer 45

Reduced TRALI Reduced non-haemolytic transfusion reactions Reduces CMV transmission Reduces GVHD

Question 46

Most common gene mutation in hereditary haemochromatosis

Answer 46

H63D mutation of the HFE gene

Question 47

Absence of CD55 and CD 59 on RBC

Answer 47

Paroxysmal nocturna haemaglobinuria

Question 48

Triad of PNH

Answer 48

1. Haemolytic anaemia 2. Venous thrombosis (especially intraabdominal) 3. Deficient haematopoesis (often pancytopaenia) Signs occurring after infection

Question 49

PNH mechanism

Answer 49

Defective production of PIG-A which is essential for GPI anchor resulting in decreased CD55 and CD 59 on cells (protects from complement mediated lysis)

Question 50

Treatment of PNH

Answer 50

Anticoagulation if thrombosis | Eculizumab (anti-C5)

Question 51

Bites and blisters on film | X Linked

Answer 51

G6PD deficiency

Question 52

Thorny apple cells | Autosomal recessive

Answer 52

Pyruvate kinase deficiency

Question 53

Eliptocytes

Answer 53

Red cell membrane disorder

Question 54

Clinical outcomes iron overload

Answer 54

- Cardiac failure - skin pigmentation - Endocrine failure: hypopituitary, hypogonadism, infertility, delyed puberty, failure to thrive, groath retardatin, diabetes, OP

Question 55

Clinical outcomes B thalassaemia

Answer 55

Severe anaemia - require transfuions Hepatosplenomegaly Iron overload - require chelation

Question 56

Good prognostic features AML

Answer 56

``` NPM + CEBPA + t(15:17) t(8:21) Inversion q16 ```

Question 57

Bad prognostic features AML

Answer 57

cKIT FLT3 + Del7q

Question 58

Treatment for AML that targets FLT-3

Answer 58

Midostaurin - kinase inhibitor

Question 59

APML genetics

Answer 59

t(15:17) | PML-RARa

Question 60

CML genetis

Answer 60

t(9:22) BCR-ABL

Question 61

MPD genetics

Answer 61

JAK-2 V617F mutation

Question 62

MDS genetics (good prognosis)

Answer 62

Deletion 5q | SPARC and RPS14 deficiency

Question 63

Treatment of CML

Answer 63

Tyrosine Kinase inhibitors - imatinib (effusion/oedema) - dasitinib (DM, long Qt, vascular events) - nilotinid (PAH)

Question 64

Good prognostic factor for ET/Primary myelofibrosis

Answer 64

CALR

Question 65

Treatment of PRV

Answer 65

Phlebotomy until haematocrit <45% | Low dose aspirin

Question 66

Cytogentic abnormality associated with response to lenolidamide in MDS

Answer 66

Deletion 5q

Question 67

HHV8 cancer-associated

Answer 67

Primary effusion lymphoma

Question 68

Hep C cancer association

Answer 68

Splenic marginal lymphoma

Question 69

Burkitt's lymphomac cytogenetics

Answer 69

t (8:14) translocation

Question 70

Mantle cell lymphoma cytogenetics

Answer 70

t(11:14) translocation = cyclin D1

Question 71

Follicular lymphoma cytogenetics

Answer 71

t(14:18) translocation = BCL-2

Question 72

Treatment high-grade NH lymphoma

Answer 72

Radiotherapy RCHOP Rituximab if CD-20 positive Transplant if relapse

Question 73

Treatment of low-grade NHL

Answer 73

RTx | Single agent chemo

Question 74

RCHOP

Answer 74

``` Rituximab Cyclophosphamide Hydroxydaunrubacin Vincristine Prednisolone ```

Question 75

Hodgkin Lymphoma treatment

Answer 75

ABVD chemo RTx AutoSCT. ifrelapsed AlloSCT if relapse post AUto

Question 76

ABVD agents

Answer 76

Doxorubicin Bleomycin Vinblastine Dacarbazine

Question 77

CLL novel therapies

Answer 77

Obinutuzumab - mAB CD2- Ibrutinib - B-TK inhibitor Venetoclax - Inhibits Bcl-2 (TLS risk) Idelalisib - PI3Kd inhibitor

Question 78

Ig subclassess involved in MM

Answer 78

IgG>IgA>IgM (waldenstroms)

Question 79

Bortezemib MOA/Indication / SE

Answer 79

MOA: Proteasome inhibitor Indication: MM SE: Peripheral neuropathy, diarrhoea, thrombocytopaenia

Question 80

CLL film

Answer 80

Smear cells

Question 81

When to consider treating CLL

Answer 81

Lymphocytes doubling in <6 months Symptoms Stage 3 - anaemia Stage 4 - thrombocytopaenia

Question 82

Coeliac cancer association

Answer 82

T Cell lymphoma

Question 83

Treatment of ET

Answer 83

Aspirin | Consider cytoreduction if plt >1000

Question 84

Cells involved in haemophagocytic lymphohistiocytosis

Answer 84

cytotoxic T cells & Natural Killer (NK) cells.