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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
Which cytogenetic factors would you expect to see in CML?
Philadelphia chromosome positive BCR-ABL translocation
26
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.
Nilotinib - SE CV, prolonged QTc Dasatinib - SE pleural effusions Ponatinib - SE CV (recommended in some mutations e.g. T3151)
27
What cytogenetic factors help to differentiate mantle cell lymphoma from CLL? Name 2.
CD5 +ve CD23 -ve
28
What is Binet staging for CLL?
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
29
What is standard treatment for CLL?
Rituximab + fludarabine/obinutuzumab + cyclophosphamide For p17-/p53 patients - ibrutinib in addition to above
30
What are typical side effects of ibrutinib? Name 3.
Atrial fibrillation Diarrhoea Platelet dysfunction
31
How does hairy cell leukaemia typically present? Name 3 features.
Pancytopenia Splenomegaly No lymph nodes
32
What markers would you expect to see in hairy cell leukaemia? Name 4.
CD19 and CD 20 (B-cell) CD25 (IL-2 receptor) CD103
33
How is hairy cell leukaemia treated?
Single 7 day course of cladrabine If BRAF +ve - consider vemurafenib
34
What are the 4 “classical” subtypes of Hodgkin’s lymphoma?
Nodular sclerosing - most common Mixed cellularity Lymphocyte rich - best prognosis Lymphocyte depleted
35
What is Ann Arbor staging for Hodgkin lymphoma?
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
How do you treat Hodgkin’s lymphoma?
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
Which translocation if involved in the diagnosis of Burkitt’s lymphoma?
T(14;18) - c-Myc
38
What are the two different types of cutaneous T-cell lymphoma? Name 2
Mycosis fungoides - initially flat eczematous lesions becoming raised Sezary syndrome - related to mycosis fungoides, but involving all the skin with blood involvement
39
How do you treat cutaneous T-cell lymphoma?
Photopheresis Alemtuzumab (anti-CD52)
40
How is myelofibrosis treated?
JAK2 inhibitors Ruxolitinib can be given to both JAK2 +ve and -ve patients
41
Name 5 complications of polycythaemia rubra Vera.
Aqua-pruritis Thrombosis Bleeding Myelofibrosis AML
42
How is PRV treated?
Aspirin - reduced risk of thrombosis Venesection - best if neuts/plts = N Hydroxyurea if high counts Keep PCV <0.45
43
In myeloproliferative neoplasms, what confers a poor prognosis?
Being “triple negative” - JAK2, calreticulin and MPL
44
What do you look for in terms of surface markers to diagnosis paroxysmal nocturnal haemoglobinuria?
LACK of CD55 and CD59 on RBCs/neutrophils/monocytes
45
What is the pathophysiology of paroxysmal nocturnal haemoglobinuria?
Clone lacking GPI anchor proteins on surface membranes of RBC/neutrophils, increased susceptibility to complement NO depletion in microcirculation
46
How in PNH treated?
Eculizumab (anti-C5)
47
How do the different levels of gene deletion present in alpha thalassaemia?
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
How does beta thalassaemia manifest itself?
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
Name 5 features you would expect to see in autoimmune haemolytic anaemia.
Anaemia Reticulocytes Bilirubin (unconjugated) Low haptoglobin (used up removing excess Hb) Direct antiglobulin test (i.e. Coomb’s)
50
In hereditary spherocytosis, in which proteins would you expect mutations to occur? Name 4.
Ankyrin Band 3 Spectrum Protein 4.2
51
What types of cells would you expect to see in G6PD deficiency? Name 3.
Bite cells Keratocytes Heinz bodies
52
What is the pathophysiology of G6PD?
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
Name 5 drugs/drug groups that are implicated in drug-induced oxidative haemolysis.
Sulphonamides Dapsone Antimalarials Co-trimoxazole Naphthalene
54
Name 3 things that can result in cold agglutinins.
EBV Mycoplasma Lymphoma Cold agglutinin disease (cold agglutinins + haemolysis) are strongly associated with lymphoma.
55
Name 5 causes of acquired von Willebrand syndrome.
Aortic stenosis and LV assist devices Essential thrombocythaemia - vWF consumed by very high platelet numbers Immune-mediated Malignancy Hypothyroidism
56
What are the three different types of Von Willebrand disease?
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
Which blood type is more likely to result in lower levels of VWF?
Blood type O
58
Name 2 ways of measuring antiphospholipid antibodies.
1. Lupus anticoagulant testing | 2. Enzyme immunoassay (anticardiolipin abs, b2-glycoprotein I antibodies)
59
Name 5 drugs/drug groups that are implicated in drug-induced oxidative haemolysis.
Sulphonamides Dapsone Antimalarials Co-trimoxazole Naphthalene
60
Name 3 things that can result in cold agglutinins.
EBV Mycoplasma Lymphoma Cold agglutinin disease (cold agglutinins + haemolysis) are strongly associated with lymphoma.
61
Name 5 causes of acquired von Willebrand syndrome.
Aortic stenosis and LV assist devices Essential thrombocythaemia - vWF consumed by very high platelet numbers Immune-mediated Malignancy Hypothyroidism
62
What are the three different types of Von Willebrand disease?
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
Which blood type is more likely to result in lower levels of VWF?
Blood type O
64
Name 2 ways of measuring antiphospholipid antibodies.
1. Lupus anticoagulant testing 2. Enzyme immunoassay (anticardiolipin abs, b2-glycoprotein I antibodies) B2-glycoprotein is the major pathogenic antibody
65
Name 2 ways of testing of for lupus anticoagulant.
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
What is a mixing study, and how is it interpreted?
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
Name 3 major defects and 2 minor defects that result in familial thrombophilia.
``` Major 3 (heterozygotes affected) Antithrombin deficiency Protein C deficiency Protein S deficiency ``` Minor 2 Factor V Leiden Prothrombin variant
68
Which factors are affected in haemophilia A and B respectively?
Haemophilia A - Factor VIII Haemophilia B - Factor XI
69
How is haemophilia A treated? Name 3 methods.
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
Name 4 conditions that can cause an isolated bilirubin elevation.
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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