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GCUH FRACP Writtens 2020 > Haematology > Flashcards

Haematology Flashcards

1
Q

What treatments can you give for HITS?

A

Direct thrombin inhibitors - bivalirudin, argatroban
Danaparoid
Fondaparinux

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2
Q

How do you diagnose progressive myeloma (symptomatic)? 2 criteria required.

A

Paraprotein in serum +/- urine

Evidence of end organ damage

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3
Q

How do you diagnose asymptomatic myeloma? 2 criteria required.

A

Serum paraprotein >30g/L

+/- bone marrow clonal plasma cells >10%

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4
Q

What are the staging criteria for multiple myeloma?

A

Stage I - B2-microglobulin < 3.5, albumin >35

Stage III - B2-microglobulin > 5.5, albumin any level

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5
Q

Which translocations confer a poorer prognosis in multiple myeloma?

A

T(4;14)

T(14;16)

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6
Q

Which translocation confers a more favourable prognosis in multiple myeloma?

A

T(11;14)

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7
Q

How is multiple myeloma treated in non-transplant candidates?

A

Lenalidomide + dexamethasone

Then bortezomib and melphalan/cyclophosphamide and prednisone

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8
Q

How is multiple myeloma treated in transplant candidates (>70)?

A

Induction: chemo + bortezomib + dexamethasone for 3-4 months

Then melphalan + SCT

Maintenance is steroids + thalidomide/lenalidomide

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9
Q

How is multiple myeloma treated in transplant candidates (<70)?

A

Bortezomib induction

Auto SCT

Maintenance: thalidomide/lenalidomide +/- prednisolone + bisphosphonate

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10
Q

What is the most common type of myeloma?

A

IgG in 50-65% of cases

Followed by IgA (20-25%, do less well), light chain (15-20%) and then IgM/IgD at approx 1-2%

2-3% non-secretory

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11
Q

Which type of patients are more likely to progress from MGUS to myeloma? 3 considerations.

A

IgA paraprotein more likely to progress than IgG

If serum free light chain ratio is abnormal, more likely to progress

High levels of paraprotein e.g. >15g/L

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12
Q

What are treatment criteria for treatment of MM? 3 criteria.

A

Raised calcium, renal impairment, anaemia and bone pain AND

2 lesions on MRI/low dose CT scan OR

Plasma cell >60 in bone marrow OR

SFLC ratio >100

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13
Q

What are typical side effects of thalidomide? Name 3.

A

Constipation

Thrombosis

Permanent neuropathy

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14
Q

Which are the highest yield areas to biopsy for AL amyloidosis?

A

Kidney/liver biopsy (>90%)

Fat biopsy (70-80%)

Rectal (50-70%)

BM looking at blood vessel walls (50-55%)

BM + fat = >90%

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15
Q

Which cytogenetic factors confer a poor prognosis in AML? Name 3.

A

Deletion chromosome 7q

Inversion q3 or t(3;3)

Multiple abnormalities simultaneously

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16
Q

Which cytogenetic factors confer a favourable prognosis in AML? Name 3.

A

T(15;17) - M3

Inversion q16 (monocytic, with eosinophilia)

T(8;21) (often M2)

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17
Q

What is Venetoclax and what can it be used in? Not sure if this will be tested.

A

BCL2 inhibitor

Being trialled in CLL/LG NHL (for CLL, good results with obinutuzumab)

Shows promise for NPM1 patients

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18
Q

In which type of AML are patients more likely to have soft tissue involvement, particularly skin and gums?

A

Monocytic (M4-5)

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19
Q

ATRA is used in the treatment of APML. What is ATRA syndrome, and how is it treated?

A

Release of cytokines, causing drop in O2/ARDS

Treated with dexamethasone

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20
Q

What are the current treatment options available for APML? Name 2.

A

ATRA

Arsenic - superior to ATRA/chemo for upfront treatment
- SE includes Torsades de Points

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21
Q

What does T-cell ALL frequently present with?

A

Mediastinal involvement.

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22
Q

Name 5 poor prognostic features in ALL.

A

Philadelphia chromosome t(9;22) positive

BCR-ABL fusion gene positive

High WCC

Extra-medullary disease - CNS, testes

Age - poorer in older adults, and in infants

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23
Q

What is the treatment course for ALL?

A

1 month induction - anthra/vincristine/steroid/asparaginase

1 month consolidation - cyclophosphamide/Ara-C/etoposide

1 month intensification - HD MTX or cranial XRT

Re-induction 1 month and then re-consolidation 1 month

Oral maintenance to complete 2-3 year treatment

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24
Q

In which patients is allogenic transplant indicated for ALL? Name 2 groups.

A

Adult patients in 1st complete remission if high risk

If <35 (following 1st complete remission)

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25
Q

Which cytogenetic factors would you expect to see in CML?

A

Philadelphia chromosome positive

BCR-ABL translocation

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26
Q

In patients that are imatinib resistant/intolerant for CML, what are the other treatments you could consider, and what are their side effects? Name 3.

A

Nilotinib - SE CV, prolonged QTc

Dasatinib - SE pleural effusions

Ponatinib - SE CV (recommended in some mutations e.g. T3151)

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27
Q

What cytogenetic factors help to differentiate mantle cell lymphoma from CLL? Name 2.

A

CD5 +ve

CD23 -ve

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28
Q

What is Binet staging for CLL?

A

Stage A - <3 LN groups and Hb and platelets >100

Stage B - 3 or more sites involved, Hb and platelets >100

Stage C - Hb <100 or platelets <100

Stage predicts outcome; stage A only requires treatment if systemic symptoms

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29
Q

What is standard treatment for CLL?

A

Rituximab + fludarabine/obinutuzumab + cyclophosphamide

For p17-/p53 patients - ibrutinib in addition to above

30
Q

What are typical side effects of ibrutinib? Name 3.

A

Atrial fibrillation

Diarrhoea

Platelet dysfunction

31
Q

How does hairy cell leukaemia typically present? Name 3 features.

A

Pancytopenia

Splenomegaly

No lymph nodes

32
Q

What markers would you expect to see in hairy cell leukaemia? Name 4.

A

CD19 and CD 20 (B-cell)

CD25 (IL-2 receptor)

CD103

33
Q

How is hairy cell leukaemia treated?

A

Single 7 day course of cladrabine

If BRAF +ve - consider vemurafenib

34
Q

What are the 4 “classical” subtypes of Hodgkin’s lymphoma?

A

Nodular sclerosing - most common

Mixed cellularity

Lymphocyte rich - best prognosis

Lymphocyte depleted

35
Q

What is Ann Arbor staging for Hodgkin lymphoma?

A

Stage I - single lymph node group

Stage II - two separate lymph nodes or organ both confined to one side of the diaphragm

Stage III - both sides of the diaphragm

Stage IV - extra-lymphatic organ involvement

B symptoms - weight loss of >10% or night sweats

36
Q

How do you treat Hodgkin’s lymphoma?

A

Earlier stage - ABVD (adriamycin, bleomycin, vinblastine, dacarbazine)

Later - BEACOPP (bleo, etoposide, adria, cyclo, vincristine, procarbazine, pred)

Brentuximab is a recent anti-CD30; checkpoint inhibitors under consideration

37
Q

Which translocation if involved in the diagnosis of Burkitt’s lymphoma?

A

T(14;18) - c-Myc

38
Q

What are the two different types of cutaneous T-cell lymphoma? Name 2

A

Mycosis fungoides - initially flat eczematous lesions becoming raised

Sezary syndrome - related to mycosis fungoides, but involving all the skin with blood involvement

39
Q

How do you treat cutaneous T-cell lymphoma?

A

Photopheresis

Alemtuzumab (anti-CD52)

40
Q

How is myelofibrosis treated?

A

JAK2 inhibitors

Ruxolitinib can be given to both JAK2 +ve and -ve patients

41
Q

Name 5 complications of polycythaemia rubra Vera.

A

Aqua-pruritis

Thrombosis

Bleeding

Myelofibrosis

AML

42
Q

How is PRV treated?

A

Aspirin - reduced risk of thrombosis

Venesection - best if neuts/plts = N

Hydroxyurea if high counts

Keep PCV <0.45

43
Q

In myeloproliferative neoplasms, what confers a poor prognosis?

A

Being “triple negative” - JAK2, calreticulin and MPL

44
Q

What do you look for in terms of surface markers to diagnosis paroxysmal nocturnal haemoglobinuria?

A

LACK of CD55 and CD59 on RBCs/neutrophils/monocytes

45
Q

What is the pathophysiology of paroxysmal nocturnal haemoglobinuria?

A

Clone lacking GPI anchor proteins on surface membranes of RBC/neutrophils, increased susceptibility to complement

NO depletion in microcirculation

46
Q

How in PNH treated?

A

Eculizumab (anti-C5)

47
Q

How do the different levels of gene deletion present in alpha thalassaemia?

A

One gene deletion - silent carrier (aa/a or aa/a-)

Two gene deletion - alpha trait (a-/a- or aa/—)

Three gene deletion - HbH disease (a-/—)

Four gene deletion - Barts hydros fetalis (—/—)

48
Q

How does beta thalassaemia manifest itself?

A

Increased HbA2 (a2d2)

Trait (heterozygous) - asymptomatic, mild anaemia, MCV <72, HbA2 3.6% - 5% (normally <3.5%)

Major - Hb 30-70, MCV 50-70, expanded bone marrow and bony deformities, hyposplenism, iron overload resulting in cardiac failure and arrhythmias, delayed growth and puberty, transfusion dependent

49
Q

Name 5 features you would expect to see in autoimmune haemolytic anaemia.

A

Anaemia

Reticulocytes

Bilirubin (unconjugated)

Low haptoglobin (used up removing excess Hb)

Direct antiglobulin test (i.e. Coomb’s)

50
Q

In hereditary spherocytosis, in which proteins would you expect mutations to occur? Name 4.

A

Ankyrin

Band 3

Spectrum

Protein 4.2

51
Q

What types of cells would you expect to see in G6PD deficiency? Name 3.

A

Bite cells

Keratocytes

Heinz bodies

52
Q

What is the pathophysiology of G6PD?

A

Hydrogen peroxide is normally converted to water and oxygen

Lack of G6PD means hydrogen peroxide results in oxidant radicals,which damage Hb and membrane, leading to formation of Heinz bodies

53
Q

Name 5 drugs/drug groups that are implicated in drug-induced oxidative haemolysis.

A

Sulphonamides

Dapsone

Antimalarials

Co-trimoxazole

Naphthalene

54
Q

Name 3 things that can result in cold agglutinins.

A

EBV

Mycoplasma

Lymphoma

Cold agglutinin disease (cold agglutinins + haemolysis) are strongly associated with lymphoma.

55
Q

Name 5 causes of acquired von Willebrand syndrome.

A

Aortic stenosis and LV assist devices

Essential thrombocythaemia - vWF consumed by very high platelet numbers

Immune-mediated

Malignancy

Hypothyroidism

56
Q

What are the three different types of Von Willebrand disease?

A

Type 1 - reduced level of VWF protein (<30 IU/dL or <0.3 IU/mL)

Type 2 - reduced function

Type 3 - very low levels (both alleles affected)

57
Q

Which blood type is more likely to result in lower levels of VWF?

A

Blood type O

58
Q

Name 2 ways of measuring antiphospholipid antibodies.

A
  1. Lupus anticoagulant testing

2. Enzyme immunoassay (anticardiolipin abs, b2-glycoprotein I antibodies)

59
Q

Name 5 drugs/drug groups that are implicated in drug-induced oxidative haemolysis.

A

Sulphonamides

Dapsone

Antimalarials

Co-trimoxazole

Naphthalene

60
Q

Name 3 things that can result in cold agglutinins.

A

EBV

Mycoplasma

Lymphoma

Cold agglutinin disease (cold agglutinins + haemolysis) are strongly associated with lymphoma.

61
Q

Name 5 causes of acquired von Willebrand syndrome.

A

Aortic stenosis and LV assist devices

Essential thrombocythaemia - vWF consumed by very high platelet numbers

Immune-mediated

Malignancy

Hypothyroidism

62
Q

What are the three different types of Von Willebrand disease?

A

Type 1 - reduced level of VWF protein (<30 IU/dL or <0.3 IU/mL)

Type 2 - reduced function

Type 3 - very low levels (both alleles affected)

63
Q

Which blood type is more likely to result in lower levels of VWF?

A

Blood type O

64
Q

Name 2 ways of measuring antiphospholipid antibodies.

A
  1. Lupus anticoagulant testing
  2. Enzyme immunoassay (anticardiolipin abs, b2-glycoprotein I antibodies)

B2-glycoprotein is the major pathogenic antibody

65
Q

Name 2 ways of testing of for lupus anticoagulant.

A

Sensitive APTT - antiphospholipid Abs prolong APTT-type test

Dilute Russell’s viper venom test (dRVVT) - venom activates Factor X - blocked by antiphospholipid antibodies

Positivity for >1 test is associated with thrombosis

66
Q

What is a mixing study, and how is it interpreted?

A

Half a normal plasma pool is added to patient’s blood sample

50% of coagulation factors are sufficient for normal clotting times

There, if a factor deficiency is present, the prolonged APTT will correct

However, if the sample contains an inhibitor (e.g. heparin, antiphospholipid ab etc.) the admixed plasma will be inhibited

67
Q

Name 3 major defects and 2 minor defects that result in familial thrombophilia.

A 
Major 3 (heterozygotes affected)
Antithrombin deficiency
Protein C deficiency
Protein S deficiency

Minor 2
Factor V Leiden
Prothrombin variant

68
Q

Which factors are affected in haemophilia A and B respectively?

A

Haemophilia A - Factor VIII

Haemophilia B - Factor XI

69
Q

How is haemophilia A treated? Name 3 methods.

A

Activated prothrombin complex concentrate

Recombinant factor VIIa (Novoseven) - bypassing need for factor VIII

Emicizumab - a bispecific antibody that mimics FVIII activity by binding to both FIX and X - used to treat patients with haemophilia A with developed inhibitors to FVIII

70
Q

Name 4 conditions that can cause an isolated bilirubin elevation.

A

Gilbert’s syndrome (uridine diphosphoglucuronate-glucuronosyltransferase 1A1)

Crigler-Najjar Syndrome (unconjugated)

Rotor and Dubin-Johnson syndromes (conjugated)

Haemolysis (unconjugated)

GCUH FRACP Writtens 2020 (14 decks)

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