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RACP Written Exam 2021 > Haematology > Flashcards

Haematology Flashcards

1
Q

Therapy related AML/MDS

A

1) Alkylating agents
- Cyclophosphamide
- Melphalan
- Busulfan
2) Topoisomerase II inhibitors
- Etoposide (testicular cancer)
- Mitoxantrone

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2
Q

EBV and lymphomas

A

Predominantly in the immunocompromised host

  • Burkitt
  • Hodgkins
  • DLBCL
  • PCNSL
  • Plasmablastic lymphoma
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3
Q

BCR ABL - Philadelphia chromosome

A

translocation 9:22 –> deregulated tyrosine kinase activity

Note PCR of BCR-ABL can be used to monitor for molecular response.

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4
Q

Polycythemia driver genes

A

1) JAK 2
- V617F (96%)
- exon 12 (4%)

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5
Q

Lab findings in PCV

A

1) Elevated HB (16.5 male/16.0 female)
2) Suppressed endogenous EPO
3) Iron deficiency (functional iron deficiency)

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6
Q

Low risk PCV

A

1) <65
2) No hx associated arterial or venous thrombosis

Aspirin only

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7
Q

High risk

A

1) >65
2) Prior PV associated arterial or venous thrombosis

Aspirin 
Cytoreductive therapy 
- Venesection 
- Hydroxeurea 
- Interferon
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8
Q

Essential thrombocytopenia

A

1) Jak 2 (60-65%)
2) CALR
3) MPL

Triple negative = low risk of vascular events

Mgmt Aspirin

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9
Q

Primary myelofibrosis genetics chanegs

A

1) Jak 2 (60-65%)
2) CALR
3) MPL

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10
Q

Clinical findings for Primary Myelofibrosis

A

1) Anaemia
2) Leukocytosis
3) raised LDH
4) Splenomegaly

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11
Q

Management of PM

A

1) Supportive
2) JAK 2 inhibitors (ruxolitinib)
3) Allogenic stem cell transplant

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12
Q

Treatment for CML

A

1) TKI: imatanib, dasatanib

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13
Q

Main adverse effect of dasatanib

A

Pleural and pericardial effusions

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14
Q

CMML Chronic myelomonocytic leukaemia

A

1) Leukocytosis + monocytosis

2) Absence of driver mutations (JAK2/BCR-ABL)

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15
Q

Treatment for CMML

A

1) Supportive
2) Cytoreductive therapy i.e hydroxyurea
3) Hypomethylating agents i.e azacitadine

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16
Q

What do you treat 5q deletion MDS with?

A

Lenolidamide

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17
Q

MDS treatment (non-5q) for poor ECOG/older patients

A

1) Tranfusion support
- Blood transfusion
- Platelet tranfusion
2) Growth factors
- Erythropoiesis stimulating agents
- Granulocyte colony stimulating factor

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18
Q

GATA 2 germline mutation (not complete)

A

Note all will develop MDS therefore early transplant

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19
Q

Disease modifying agents for low risk MDS

A

1) Activin receptor type II ligand traps: Luspatercept/Sotatercept

MOA; fusion protein that blocks transforming growth factor - TGF B- that inhibits erythropoiesis

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20
Q

Disease modifying agents in high risk MDS

A

1) Azacitadine: hypomethylating agent

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21
Q

AML diagnosis

A

1) Pancytopenia
2) > 20% blasts in aspirate
3) Blasts with Bauer rods

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22
Q

Flt 3 ITD mutation in AML good or poor prognosis

A

Confers poor prognosis

NPM1 + FLT 3 = better prognosis

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23
Q

Treatment for AML (low ECOG)

A

Induction
3 days anthracycline
7 days cytarabine

Allogenic stem cell transplant

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24
Q

Treatment for AML (high ECOG or age >70)

A

Low dose cytarabine

Azactiadine - less than 30% blasts (due to slow cytoreduction)

25
Q

Targeted therapies in AML

A

1) FLT 3 mutation: Midostaurin

2) ABT-199: Venetoclax + Azacitadine

26
Q

APML

A

PML RARA fusion –> blockage of pro-myelocytes non differentiation and no maturation
15:17

27
Q

Blood film findings on APML

A

Bilobed nucleoli

Hypo/hyperganulated cytoplasma

28
Q

Treatment for APML

A

1) Atra –> degrades fusion gene –> allows differentiation 2) Arsenic
3) Identify and monitor for DIC

29
Q

ALL diagnostic criteria

A

20% blasts in bone marrow
No auer rods in blasts

Nb immunophenotyping via flow cytometry

30
Q

Poor prognostic factors in ALL

A 
B-ALL
- BCR-ABL 
- Philadelphia like (no BCR-ABL) 
T-ALL
- Early thymocyte precursor phenotype

In all lineages older age = poorer prognosis

31
Q

Chronic lymphocytic leukaemia diagnosis

A

1) Lymphocytosis (B-cell lymphocytes) +/- cytopenias

2) CD5 and CD19 clonal B cells

32
Q

Good prognosis in CLL

A

13q deletion (“the lucky deletion)

33
Q

Poor prognosis in CLL

A

11q/17p deletion
Zap-70
CD38+
Unmutated heavy chain gene

34
Q

When to treat CLL

A

1) Cytopenias
2) Lymphocyte doubled in <6 months or >50% increase in 2 months
3) Disease related symptoms
4) Autoimmune complications

35
Q

Richters transformation in CLL

A

Tranformation to lymphoma ie DLBCL

36
Q

Treatment CLL

A

1) Without deletion of 17p (TP53 mutation)
- Chemotherapy
2) With deletion of 17p
- Immunotherapy; Ibrutinib/Venetoclax

37
Q

MOA of Venetoclax

A

Inhibits BCL-2 which is an anti-apoptotic protein that is over expressed in CLL

38
Q

Diagnosis of Systemic AL Amyloidosis

A

Biopsy with Congo red stain

39
Q

Criteria for Myeloma

A

1) Bone marrow >10% clonal plasma cells
2) End organ damage
- CRAB
- SLIM

40
Q

SLIM criteria for MM

A

S: Sixty percent of=r greater clonal plasma cells on bone marrow
LI: Free Light chain ratio >100
M: MRI demonstrate one focal lesion that is at least 5mm or greater in size

41
Q

Prognostic markers for MM

A 
B2 microglobulin
< 3.5 good
> 5 bad 
Albumin
> 35 good
< 35 bad
42
Q

Main adverse effects of carlifilzomib

A

Heart failure

43
Q

Pathogenesis of Burkitt Lymphoma

A

Translocation of the MYC oncogene from chromosome 8

  • T(8:14)
  • T(2:8)
  • T(8:22)
44
Q

Follicular Lymphoma translocation

A

T14:18

Leads to BCL over expression

45
Q

GELF criteria for commencement of chemo-immunotherapy

A

Symptomatic disease
Threatened end organ damage
Bulky disease

46
Q

Which gene mutation has been identified in WM

A

MYD88

47
Q

Complications associated with IGM MGUS

A

1) Perhiperal neuropathy
2) Cryoglobulinaemia
3) Hyperviscosity syndrome
4) Malabsorption - from depositing in GIT

48
Q

Histological findings in Hodgkins Lymphoma

A

1) Reed Sternberg cells

2) CD30 positive

49
Q

Brentuximab MOA and use

A

Anti-CD30

Used for relapsed refractory Hodgkins lymphoma

50
Q

Emicizumab Mechanism of action

A

Monoclonal antibody that binds activated IX to X thus bypassing the need of activated factor VIII

Used for haemophilia A

51
Q

What does prothrombinex contain in Australia?

A

coagulation factors II, IX and X and low levels of factors V and VII

52
Q

TTP

A

Headaches

Plasmapharesis

53
Q

HLH

A

Elevated Ferritin

Haemophagocytosis on blood film

54
Q

Felty’s

A

RA/Splenomegaly/Neutropenia

55
Q

Spur cell haemolytic anaemia

A

Cholesterol deposition in RBC cell membranes from Apo-lipoprotein A2
Spur cell –> Extravascular Haemolysis

56
Q

B12 Deficiency

A

Megaloblastic Anaemia
Hyper-segmented neutrophils

Post gastrectomy common

57
Q

Promthrombinex inclusion

A

2/7/9/10

58
Q

Erlenmyer Flask Abnormality

A

Gaucher Disease (Inherited disease of storage)

59
Q

CART cells

A

CD 19

Cytokine release syndrome

RACP Written Exam 2021 (18 decks)

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