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RACP 2022 Written > Haematology > Flashcards

Haematology Flashcards

1
Q

Translocation associated with mantle cell lymphoma

A

(11;14) translocation

(q13;q32) cyclin D1 (CCDN1) and immunoglobulin heavy chain (IgH)

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2
Q

List 2 GIIB/IIIB inhbitors

A

abciximab
eptifibatide

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3
Q

Deficiency in:
- Bernard soulier
- grey platelet syndrome
- glanzmans thrombesthenia

A

BS - glycoprotein abnormalities
GPS - alpha granule deficiency
GT - GIIb/IIIB defect

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4
Q

Treatment of essential thrombocytosis

A

If no JAK mutation, age <60yo, no vasomotor sx or CVS risk factors, can observe

Otherwise, aspirin daily (BD if CVS risk factors)

ADD hydroxyurea if high risk (i.e. previous clots, >60YO) with target platelets <400

ADD anticoagulation if previous VTE

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5
Q

What conditions is eculizumab inducated for?

A

PNH
Atypical HUS (complement mediated TMA)

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6
Q

Hepcidins action on iron

What effect does inflammation have on it?

A

Decreases serum iron

Inhibits ferroportin (major role)
- decreased recycling of iron from old RBCs/macs via the reticuloendothelial system
- decreased release of iron into the circulation at basolateral membrane of enterocytes

Inhibits intestinal iron absorption (minor role

Inflammation increases hepcidin

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7
Q

Common mutations in myelodysplastic syndromes

Which are associated with a poor prognosis?

A

TET2
SRSF2
ASXL1
DNMT3A
RUNX1

SRSF2, RUNX1 and TP53 = poor prognosis

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8
Q

Which form of MDS does lenalidomide work best in

A

isolated del5q

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9
Q

Poor prognostic markers in MM

A

High beta 2 microglobulin
Low albumin
t(4;14)

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10
Q

Standard treatment for MM if ineligible for transplant

What can be added if high risk?

A

Bortezomib (proteosome inhibitor)
Melphalan (akylating agent) or lanalidomide
Prednisone

Antibodies
- antiCD38: daratunumab, isatuximab

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11
Q

Most common feature of MM

A

Anemia

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12
Q

Hodgkins lymphoma with best prognosis

worst prognosis?

A

Nodular sclerosing’

Lymphocyte depleted (worst)

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13
Q

What are Dohle bodies and when do they occur

A

Toxic granulation of neutrophils

Leukomoid rection - associated with high LAP socre and differentiating feature from CML

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14
Q

Features of WM

A

Funduscopic abnormalities
Constitutional “B” symptoms
Bleeding
Neurologic symptoms
Hyperviscosity
Lymphadenopathy
Hepatomegaly
Splenomegaly

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15
Q

What does TCT test?

Name conditions where it will be prolonged

A

Fibrinogen conversion to fibrin (i.e. the action of thrombin)

Unfractionated heparin
Direct thrombin inhibitors (dabigatran)
Liver disease
DIC and acquired fibrinogen disorders
Hypoalbuminemia
Paraproteinemia

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16
Q

Abelacimab mechanism of action

A

Factor XI inhbitor

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17
Q

Prolonged APTT with normal reptilase time indicates what

A

Heparin contamination

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18
Q

Test to distunguish secondary and primary polycythemia

A

EPO level
- low in primary
- high in secondary

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19
Q

Spindle cells and fibrosis on BM biopsy with elevate tryptase

Mutation?

A

Systemic mastocytosis

KIT mutations (D816V common)

20
Q

Empiric antibiotics for patients with febrile neutropenia life threatening penicillin allergy

Non-life threatening penicillin allergy?

A

Life threatening:
- aztreonam OR cipro
- AND vancomycin

Non-life threatening
- cefepime (no vanc)

21
Q

Empiric antibiotics for patients with febrile neutropenia and systemic compromise

A

As for non life threatening (pip-taz) and:
- ADD gentamycin
- ADD vanc if signs of cellulitis

If GI features and not on pip-taz/mero, need to cover for anaerobes with metronidazole

22
Q

What is daratunumab?
What is the major issue?

A

Anti-CD38, used in MM

Interferes with pre-transfusion cross match due to CD38 markers on RBCs

23
Q

Malignancy associated with “starry sky” picture on blood film

Mutation?

A

Burkitts Lymhoma

MYC;IgH translocation (8;14)

24
Q

What is gemtuzumab and when is it used?
Major issue?

A

Anti-CD33, used in AML (less common now)

causes hepatic sinusoidal obstructive(veno-occliusive) syndrome
Treat with defibrotide

25
Q

CML translocation

A

t(9;22)

26
Q

APML translocation

A

t(15;17)

27
Q

Indications for treatment in CLL

A

Worsening anemia/thrombocytopenia

Splenomegaly

Lymphadenopathy

Progressive lymphocytosis - doubling time <6 months OR increase >50% over 2 months

Autoimmune anemia/thrombocytopenia poorly responsive to steroids

Symptomatic/functional extranodal involvement (skin, kidney, lungs, spine)

Constitutional symptoms - unintentional LOW, fevers, fatigue, night sweats

28
Q

Poor prognostic markers in CLL

A

BIG ONE - Del(17p)/TP53

Lymphocyte doubling time <12 months
Del(11q)
Unmutated IGHV
Age >60
High B2 microglobulin

29
Q

Best evidence for prevention of post thrombotic syndrome

A

Anti-coagulation

30
Q

Definition of MM

A

> 10% clonal plasma cells + either of:

  • End organ damage (1 or more) - CRAB sx
  • presence of a biomarkers (1 or more) - ≥60% clonal plasma cells in BM, FLC ratio ≥100, MRI with >1 focal bone lesion
31
Q

Chains in:
- HbA
- HbA2
- HBF
- HBH
- Hb Barts

A

HBA: 2 alpha, 2 beta (normal)
HBA2: 2 alpha, 2 delta (beta thal)
HBF: 2 alpha, 2 gamma
HBH: 4 beta (alpha thal)
Hb Barts: 4 gamma (alpha thal)

32
Q

hemoglobin in sickle cell

A

HBS - 2 alpha, 2 beta
Mutated beta chain

33
Q

Mutation in sickle cell disease

A

missense mutation [Glu6Val, rs334] in the β-globin gene
[HBB]

34
Q

What is hemoglobin E?
When is it significant?

A

HbA chains (2 alpha, 2 beta) with mutated beta chain
Significant when occurs with beta thalasamia; significant beta chain reduction

35
Q

Features of red cell aplasia

A

Normochromic, normocytic anemia
elevated EPO
Absent erythroblasts and f giant pronormoblasts in the bone marrow.

36
Q

Microspherocytes are associated with what

A

Autoimmune hemolytic anemia

37
Q

Poor prognostic features in ALL

A

Philadelphia chromosome (9;22)
Male gender
Age <2 or >10
Initial WCC >100
CNS involvement
Non-caucasian
FAB L3
T or B cell surface markers

38
Q

Good prognostic features in ALL

A

FAB L1
Common ALL
Pre-B phenotype
Low initial WCC

39
Q

In what organ is thrombopoetin mostly produced

A

Liver
Small amounts produced in kidney and bone marrow

40
Q

Effects of anticoagulants on coagulation studies:
- Warfarin
- Apixaban
- Rivaroxaban
- Dabigatran
- Heparin

A

Warfarin
- prolonged INR, variable APTT

Apixaban, rivaroxaban
- PT>APTT, TT normal

Dabigatran
- TT>APTT>PT

Heparin
- APTT>TT, normal reptilase, normal PT

41
Q

Reason for increased VTE risk in malignancy

A

Increased tissue factor production by cancer cells –> excessive extrinsic pathway activation

42
Q

Warm Autoimmune Haemolytic Anaemia?
- Mech
- Disease Assoc
- Coombs? and BLood film
- Tx

A

IgG and complement
D: Drugs, Virus, Autoimmune, CLL
- Direct commobs anti-IgG and anti-C3 positive
- Microsphereocytes, Extravascular main in spleen
Tx: steriods, splenectomy if refractory

43
Q

Cold Autoimmune Haemolytic Anaemia?
- Mech
- Disease Assoc
- Coombs? and BLood film
- Tx

A

IgM bings + complement - destruction mainly in liver or intravascular for acute.
Tx: rituxibab
D: post infection, paroxysmal, lymphoproliferative diease
Intravscular, agglutination.

44
Q

Factor V Leiden mechanism?

A

Resitance to action of protien C factor.
Most common iherited thrombophilia.
Hetro = 5 x
Homo - 50 fold risk

45
Q

Polycythaemia rubra vera common transformations?

A

Myelofibrosis + AML
Mx: veneestction, aspirin, hydroxyurea.

RACP 2022 Written (21 decks)

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