Haem

Question 1

Management of Heparin-Induced Thrombocytopenia (HIT)

Answer 1

Switch to argatroban or danaparoid

Question 2

Indication for treatment in CLL

Answer 2

Constitutional symptoms

Bone marrow failure

Symptoms due to LN/spleen/liver elargement

Weight loss > 10% in 6 months

AI anaemia/ thrombocytopenia not responding to prednisolone

Lymphocyte doubling in <6m, or 50% increase in 2m

Question 3

Indication for use of Ibrutinib (BTK inhibitor) in CLL

Answer 3

TP53 mutation

Question 4

Pentad of features in TTP (Thrombotic Thrombocytopenic Purpura)

Answer 4

1. Thrombocytopenia
2. MAHA (anaemia + schistocytes)
3. Fever
4. Fluctuating neuro signs
5. Renal impairment

Question 5

Causes of MAHA (schistocytes seen on film)

Answer 5

TTP
HUS
DIC
Pre-eclampsia

Question 6

Presentation of HUS

Answer 6

Haemolytic anaemia (MAHA)
Thrombocytopenia
Severe AKI
Following bloody diarrhoea

Question 7

AL amyloidosis presents as

Answer 7

Nephrotic syndrome
Cardiomyopathy
Peri-orbital purpura
(Macroglossia)
(Urine free light chains)
Without features of MM

Question 8

Waldenstrom’s macroglobulinaemia (lymphoplasmacytoid malignancy) presents as

Answer 8

Monoclonal IgM paraproteinaemia
Hyperviscosity
Systemic upset

Question 9

Commonest initial treatment for CLL

Answer 9

Fludarabine + cyclophosphamide + rituximab (FCR)

Question 10

Other options for CLL treamtent, when FCR not appropriate

Answer 10

Bendamustine
Chlorambucil
Anti-CD20 (obinutuzumab)

Question 11

Management of AI anaemia/thrombocytopenia in CLL

Answer 11

Prednisolone

Question 12

Treatment of Waldenstrom macrogolubulinaemia

Answer 12

Cyclophosphamide + dexamethasone + rituximab

Question 13

Features of monoclonal gammopathy of unknown significance (MGUS)

Answer 13

Paraproteinaemia
Asymptomatic/neuropathy
Normal immune function
No bone lesions/renal impact

Question 14

Schnitzler’s syndrome

Answer 14

Chronic urticaria

Monoclonal raised IgM

Question 15

Features of methaemoglobinaemia

Answer 15

Cyanosis
Low O2 sats with normal PaO2
Ischaemia

Question 16

R-CHOP is

Answer 16

Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone

Question 17

Management of NHL - low-grade and asymptomatic

Answer 17

Watch and wait

Vaccinate (flu, haemophilus, pneumococcus, meningococcus)

Question 18

Chemo options for NHL

Answer 18

R-CHOP

FCR

Question 19

Management of primary CNS lymphoma

Answer 19

High-dose methotrexate + dexamethasone

Question 20

Imatinib is chemotherapy agent used in

Answer 20

CML
MALT-lymphoma
c-kit melanoma

Question 21

Non-Hodgkins Lymphoma with CNS involvement

Answer 21

R-CHOP + intrathecal methotrexate

Question 22

Curative treatment for myelofibrosis

Answer 22

Allogenic stem-cell transplant

Question 23

Drugs used in control of myelofibrosis

Answer 23

Hydroxyurea
IFN-a
JAK inhibitors

Question 24

Diagnostic criteria for HHT (Osler-Weber-Rendu)

Need 3 for definite diagnosis.

Answer 24

Epistaxis
Telangiectasia
AVMs or GI telangiectasia
1st-degree family history

Question 25

Impaired platelet aggregation with Ristocetin, suggests

Answer 25

von Willebrand’s disease

Question 26

BCR-ABL mutation is diagnostic for

Answer 26

CML

Question 27

BCL2 mutation seen in

Answer 27

Diffuse large B cell lymphoma

Question 28

TP53 mutation seen in

Answer 28

Diffuse large B cell lymphoma

Question 29

C-MYC mutation seen in

Answer 29

Burkitt’s lymphoma

Question 30

Commonest mutation seen in myelofibrosis

Answer 30

JAK2

Question 31

Lysosomal storage disorder which causes massive splenomegaly

Answer 31

Gaucher disease

Question 32

Lysosomal storage disorder which causes massive splenomegaly

Answer 32

Gaucher disease

Question 33

Causes of warm AIHA

Answer 33

Autoimmune (eg SLE)
CLL, lymphoma
Methyldopa

Question 34

Causes of cold AIHA

Answer 34

Mycoplasma
EBV
Lymphoma

Question 35

Prophylactic antimicrobials given when starting chemotherapy (FCR)

Answer 35

Co-trimoxazole
Aciclovir
Fluconazole

Question 36

CLL - Poor prognostic factors (median survival 3-5 years)

Answer 36

Male
>70yo
Lymphocyte count >50
Polymorphs >10%
Doubling time <12m
Raised LDH
CD38 positive
TP53 mutation

Question 37

Criteria for PCV diagnosis?

Answer 37

Major:

• Hb > 165 or hct > 49% in males/Hb> 16 or hct > 48% females
• bone marrow biopsy showing hypercellularity with prominent erythroid granulocytic and megakaryocytic prolifration
• JAK2 mutation

Minor:
*EPO below normal range

Diagnosis= 3 Major + 1 minor or first 2 Major + 1 minor as jak 2 not always present

Question 38

Rx for multiple myeloma?

Answer 38

Melphalan chemotherapy  (alters DNA guanine via alkylation) 
Bortezombib (Prevents activation of NF-kB) in patients < 65 years used as induction for those going on to high dose chemotherapy

Question 39

Criteria for MGUS?

Answer 39

M-protein < 30

<10% plasma cells

Question 40

Causes of acquired haemophilia?

Answer 40

autoimmune diseases

malginancies; pancreatic, colon, melanoma, breast

Question 41

acute chest syndrome CXR finding?

Answer 41

new pulmonary infiltrates

Question 42

culprit organisms for acute chest syndrome?

Answer 42

Pneumococcus
Mycoplasma
Chalmydia

Question 43

Poor prognostic factors for AML?

Answer 43

karyotype abnormality (e.g deletion/addition of part of chromosome 5/7), increasing age (>40), medical morbidities

Question 44

stains for acute MONOCYTIC leukaemia?

Answer 44

sudan black

MPO