ANEMIAS

1 which of the following normally contains >10% of body iron?

A Transferrin

B Heart

C Neutrophils

D Macrophage
2 Which of the following statements is NOT TRUE about sideroblastic
anaemia?

A It may be inherited

B It is most frequently caused by myelodysplasia

C It may be caused by folate deficiency

D It may respond to erythropoietin

3 Which of the following statements is NOT correct?

A A unit of blood contains 200–250 mg iron

B A man needs to absorb about 1 mg of dietary iron daily

C A molecule of transferrin may transport four atoms of iron

D Haemorrhage is the major cause of iron deficiency in the UK

4 Which ONE of the following may be used to treat iron deficiency?

A Ferric sulfate

B Desferrioxamine

C Ferrous gluconate

D Erythropoietin
5 Which ONE of the following statements regarding oral iron therapy is
TRUE?

A Slow-release preparations are indicated for patients with gastrointestinal

disorders

B Treatment should be given with vitamin C

C The maximum dose is 200 mg ferrous sulfate three times daily

D It is best given at night

E It is routinely indicated in pregnancy

6 Which ONE of these statements is TRUE about genetic haemochromatosis?

A It is dominantly inherited

B It is usually caused by mutation of the HFE gene

C It causes excess iron in the duodenal mucosa

D It is associated with reduced saturation of transferrin

7 Which ONE of the following organs is not damaged by transfusional iron
overload?

A Liver

B Kidneys

C Parathyroids

D Pituitary

E Heart
8 Which ONE of the following is a feature of iron deficiency?

A Raised serum hepcidin

B Raised serum transferrin

C Raised mean corpuscular volume (MCV)

D Hyperchromic red cells

9 In which ONE of the following is iron absorption favoured?

A Pregnancy

B Chronic inflammation

C Iron overload

D Ageing
10Which ONE of the following statements is NOT TRUE about serum iron?

A It is low in inflammatory diseases

B It is raised in iron overload

C It shows a diurnal rhythm

D It is transported in plasma by ferritin

11 Which ONE of the following is used therapeutically?

A Methylcobalamin

B Folate polyglutamate

C Hydroxocobalamin

D Methyltetrahydrofolate
12 Which ONE of the following is associated with pernicious anaemia?

A Ileal resection

B Thyroid antibodies in serum

C Pregnancy

D Malabsorption of B12–intrinsic factor complex

3 Which ONE of these statements is TRUE concerning the reduction of fola

A It needs vitamin B12

B It is inhibited by sulfonamide

C It is inhibited by methotrexate

D It occurs during thymidylate synthesis

4 Which ONE of the following does NOT cause folate deficiency?

A Antiepileptic drugs

B Veganism

C Gluten sensitivity

D Inflammation

E Pregnancy
5 Causes of microcytic red cells include which ONE of the following?

A Alcohol

B Renal disease

C α-Thalassaemia

D Increased reticulocyte count

6 Which ONE of the following statements is NOT TRUE about megaloblastic
anaemia?

A It is always caused by B12 or folate deficiency

B Bone marrow appearances are identical in B12 or folate deficiency

C It is caused by defective DNA synthesis

D It may be caused by nitrous oxide

E It is associated with jaundice

7 Which ONE of the following is caused by folate deficiency?

A Spinal cord damage

B Haemolytic anaemia

C Neural tube defect

D Duodenal atrophy
8 Which ONE of the following causes vitamin B12 deficiency?

A Haemolytic anaemia

B Veganism

C Widespread carcinoma

D Jejunal resection
9 Which ONE of the following may be caused by vitamin B12 deficiency?

A Vitiligo

B Microcytic anaemia

C Peripheral neuropathy

D Carcinoma of stomach
1 Which ONE of the following does NOT cause pancytopenia?

A Iron deficiency

B Folate deficiency

C Aplastic anaemia

D Acute myeloid leukaemia

E Cyclophosphamide
2 Which ONE of the following is NOT identified as a cause of aplastic
anaemia?

A Penicillin

B Gold

C Lead

D Chloramphenicol
3 Which ONE of the following is NOT associated with red cell aplasia?

A A raised reticulocyte count

B Parvovirus infection

C Thymoma

D Chronic lymphocytic leukaemia

4 Which ONE of these is NOT a feature of the blood count and bone marrow
in patients with aplastic anaemia?

A Abnormal ‘blast’ cells on the blood film

B Hypoplastic bone marrow with replacement by fat

C Normal appearance of neutrophils on the blood film

D Normochromic normocytic anaemia

Which ONE of these statements is NOT TRUE concerning the management of
aplastic anaemia?

A Fluorescence activated cell sorter (FACS) analysis of CD55 and CD59

expression is useful in diagnosis

B Ciclosporin is a useful agent in treatment

C Antilymphocyte globulin is of benefit in around half of cases

D Allogeneic stem cell transplantation offers only temporary remission

7 Which ONE of these statements is NOT TRUE concerning congenital
dyserythropoietic anaemias?

A The white cell and platelet counts are normal

B They show ineffective erythropoiesis and eythroblast multinuclearity

C They are often triggered by parvovirus infection

D The anaemia is usually first noted in infancy or childhood

8 Which ONE of these statements is TRUE concerning Fanconi anaemia?

A It is due to a mutation in the ATM gene

B The diagnostic test is elevated breakage after incubation of peripheral blood

lymphocytes with the DNA cross-linking agent diepoxybutane (DEB test)

C Physical development is always normal

D It has a sex-linked inheritance

.9 Which of these abnormalities is NOT a typical feature of Fanconi anaemia?

A Skeletal abnormalities of the thumbs

B Very tall stature

C Abnormal skin pigmentation

D Horseshoe kidney
Haemopoiesis
1 Which ONE of these statements is TRUE regarding normal adult bone
marrow?

A It is composed of 90% hemopoietin cells, 10% fat

B It has a myeloid: erythroid ratio of 1: 2

C It is active in the skull within adults

D It is the major source of the secretion of erythropoietin

2 Which ONE of these statements is NOT TRUE concerning the haemopoietic
stem cell?

A It is believed to represent approximately 1% of bone marrow cells

B It is thought to have a CD34+CD38− phenotype

C It resembles a small to medium sized lymphocyte

D It can be transferred between individuals during the process of stem cell

transplantation
It is thought to be rare – perhaps 1 in every 20 million nucleated cells in marrow
3 Which ONE of the following is NOT a significant component of bone marrow
stroma?

A Endothelial cells

B Adipocytes

C Fibroblasts

D Epithelial cells
4 Which ONE of these is NOT a mechanism by which granulocyte colony-
stimulating factor (G-CSF) can increase neutrophil production?

A Increased differentiation from progenitor cells

B Reduction in the self-renewal of haemopoietic stem cells

C Increased rate of maturation of immature myeloid cells

 D Suppression of apoptosis of myeloid cells
5 Which ONE of these cytokines has an important role in normal early
haemopoiesis?

A IL-1

B IL-2

C IL-3

D IL-4
6 Which ONE of these statements is TRUE regarding JAK proteins?

A Following activation, they migrate to the nucleus and bind to DNA

B The name is an abbreviation for joined activation kinase

C Mutations in JAK2 are seen in almost all patients with polycythaemia rubra
vera

D Activation of the JAK pathway generally leads to apoptosis of the cell

7 Which ONE of these statements is most accurate regarding the major sites
of haemopoiesis during development?

A It starts in liver, moves to yolk sac and then to the bone marrow

B The liver and bone marrow are equally active during gestation

C It starts in the liver and yolk sac and moves to the bone marrow after birth

D It starts in yolk sac, moves to liver and then to the bone marrow

Erythropoiesis and general aspects of anemia

1 Which ONE of these statements is TRUE about haemoglobin?

A One molecule contains one atom of iron

B It is broken down in macrophages

C It is different in children and adults

D It has a low level in blood at birth compared with adult life

2 Which ONE of these statements is TRUE about reticulocytes?

A They contain DNA not RNA

B They are raised in megaloblastic anaemia

C They are permanently raised after splenectomy

D They are raised after haemorrhage

3 Which ONE of these statements is NOT TRUE concerning erythropoietin?

A 90% of the hormone is made in the liver

B It contains a hypoxia response gene

C One stimulus to production is a low atmospheric oxygen level

D Levels in blood are high if a tumour secreting erythropoietin is causing

polycythaemia but are low in severe renal disease or polycythaemia vera
4 Which ONE of these statements is TRUE concerning the therapeutic use of
erythropoietin?

A Side-effects include hypotension and bleeding disorders

B The main indication is in patients with leukaemia

C Oral or parenteral iron is often needed to optimize the response

D Oral administration reduces the frequency of treatment

5 Which ONE of these statements is NOT TRUE concerning the biochemistry
of haemoglobin?

A Hb is 70% saturated in arterial blood

B As the haemoglobin molecule loads and unloads O2 the individual globin chains
in the haemoglobin molecule move on each other

C When O2 is unloaded the β chains are pulled apart, permitting entry of the
metabolite 2,3-diphosphoglycerate (2,3-DPG) and resulting in a lower affinity of
the molecule for O2

D The P50 (i.e. the partial pressure of O2 at which haemoglobin is half

saturated with O2) of normal blood is 26.6 mmHg
It is 95% – and 70% in venous blood.
6 Which ONE of these conditions is described by this phrase? ‘A clinical
state in which circulating haemoglobin is present with iron in the oxidized
(Fe3+) instead of the usual Fe2+ state.’

A Methaemalbuminuria

B Methaemoglobinaemia

C Congenital dyserythropoietic anaemia

D Glucose-6-phosphate dehydrogenase deficiency

7 Which ONE of these statements is NOT TRUE concerning the red cell?

A Each cell travels an estimated 480 km in its lifetime

B It has a maximum diameter of 3.5 μmol so that it can pass through vessels of
this size

C It generates energy as adenosine triphosphate (ATP) by the anaerobic

glycolytic (Embden–Meyerhof) pathway

D It generates reducing power as NADH by the Embden–Meyerhof pathway

and as reduced nicotinamide adenine dinucleotide phosphate (NADPH) by the
hexose monophosphate shunt
8 Which ONE of these statements is TRUE concerning the red cell
membrane?

A 50% of the membrane is cholesterol

B Spectrin and ankyrin are extracellular proteins that reinforce the membrane

C Alterations in serum lipids do not influence the red cell membrane

D Inherited defects in membrane proteins can cause hereditary spherocytosis

and elliptocytosis
Hemolytic anemias
1 Spherocytes in the blood film are a feature of which ONE of the following?

A Thalassaemia major

B Autoimmune haemolytic anaemia

C Reticulocytosis

D Glucose-6-phosphate dehydrogenase (G6PD) deficiency

2 Which ONE of the following is a feature of a chronic extravascular
haemolytic anaemia?

A Raised serum conjugated bilirubin

B Low reticulocyte count

C Hypocellular bone marrow

D Gallstones
3 Which ONE of the following is NOT a cause of intravascular haemolysis?

A Rhesus incompatibility

B ABO incompatibility

C Glucose-6-phosphate dehydrogenase (G6PD) deficiency

D Red cell fragmentation syndrome

4 Which ONE of the following is NOT a feature of intravascular haemolysis?

A Jaundice

B Haemosiderin in urine

C Absent haptoglobins

D Haemoglobin in urine
5 Which ONE of these statements is TRUE regarding hereditary
spherocytosis?

A It is caused by an inherited defect in haemoglobin

B It is more common in males

C It can be treated by splenectomy

D It is more frequent in southern Europe

6 Which ONE of the following statements is NOT TRUE about autoimmune
haemolytic anaemia?

A It may be due to drugs

B It is associated with pernicious anaemia

C It may be associated with IgM antibodies in serum

D It may complicate B-cell chronic lymphocytic leukaemia

E It is associated with a positive direct antiglobulin test

7 Which ONE of the following statements is NOT TRUE about glucose-6-
phosphate dehydrogenase (G6PD) deficiency?

A It commonly presents as a chronic haemolytic anaemia

B It leads to intravascular haemolysis after certain infections

C It provides some protection against types of malaria

D Carrier females have approximately 50% G6PD levels

E It is a cause of neonatal jaundice

8 Which ONE of the following is NOT a feature of thalassaemia intermedia?

A It may be due to homozygous βo thalassaemia

B It may be associated with extramedullary haemopoiesis

C It is usually associated with splenomegaly

D It may cause iron overload

9 Which ONE of the following is used to monitor transfusion iron overload?

A Bone marrow biopsy

B Serum transferrin

C Magnetic resonance imaging

D Lung function
10 Which ONE of these statements is TRUE about β-thalassaemia major?

A The major cause of death is liver failure

B It requires iron chelation at diagnosis

C It is usually caused by deletion of β globin genes

D It may be diagnosed antenatally

11 Which ONE of these statements is TRUE about β-thalassaemia major?

A It presents at birth

B It is caused by a defect in α globin synthesis

C It is associated with splenomegaly

D It is associated with an increased risk of bone infarction

12 Which ONE of the following statements is NOT TRUE about α-
thalassaemia?

A It may cause haemoglobin H disease

B It causes a microcytic hypochromic blood picture

C It ameliorates β-thalassaemia

D It is a cause of hydrops fetalis

E It is rare except in the Far East

13 Which ONE of the following statements is TRUE about β-thalassaemia
trait?

A It is associated with a raised haemoglobin A2 level

B It is associated with iron overload

C It is associated with a reticulocytosis

D It is associated with splenomegaly

14 Which ONE of these statements is TRUE concerning sickle cell trait?

A It is a cause of anaemia

B It protects against malaria

C It occurs mainly in females

D It is a cause of frequent sickle cells in the peripheral blood

15 Which ONE of the following statements is NOT TRUE about sickle cell
anaemia?

A The oxygen dissociation curve is shifted to the right (i.e. the haemoglobin
gives up oxygen more easily than normal)

B It is associated with stunted growth

C It may cause ankle ulcers

D It is associated with stroke

E It is associated with atrophy of the spleen

16 Which ONE of the following does NOT occur frequently in children with
sickle cell anaemia?

A Splenic atrophy

B Extramedullay haemopoiesis

C Silent cerebral infarction

D Acute chest syndrome

 AL
1 Which ONE of the following statements is TRUE about acute myeloid
leukaemia?

A It is most common in children

B It is never caused by chemotherapy

C It is associated with more than 20% blast cells in the bone marrow

D Disseminated intravascular coagulation is not a presenting feature

2 Which ONE of the following statements is NOT TRUE about acute myeloid
leukaemia?

A It may cause pancytopenia

B It has a cure rate of >80% in some subtypes

C Allogeneic stem cell transplantation is needed in all patients less than 50

years old with an HLA-matched donor

D It may follow myelodysplasia

E It may cause swelling of the gums

3 Which ONE of these is least valuable in the diagnosis of acute myeloid
leukaemia?

A Immunophenotypic analysis of a bone marrow sample

B Cytogenetic analysis of peripheral blood

C Microscopic analysis of the bone marrow aspirate (morphology)

D Detection of clonal rearrangement of the immunoglobulin heavy chain gene

4 Which ONE of these is the most likely blood count in a patient who
presents with acute myeloid leukaemia?

A Hb 14 g/dL; white blood cell count (WBC) 270 × 109/L; platelets 100 × 109/L

B Hb 9 g/dL; WBC 2 × 109/L; platelets 140 × 109/L

C Hb 9 g/dL; WBC 27 × 109/L; platelets 10 × 109/L

 D Hb 14 g/dL; WBC 270 × 109/L; platelets 1000 × 109/L
5 Which ONE of the following is a common feature of all leukaemias?

A Low platelet count

B Anaemia

C Excess white cells in the bone marrow

D Enlarged spleen
6 Which ONE of these genetic abnormalities defines a patient with acute
myeloid leukaemia as having an unfavourable prognosis?

A t(8;21) translocation

B Normal karyotype

C Translocation of NPM gene

D Deletion of chromosome 7
.8 Which ONE of these statements is TRUE regarding the differentiation
syndrome seen in acute promyelocytic leukaemia?

A It can follow treatment with retinoic acid

B It is particularly associated with AML subtypes that occur secondary to

Down syndrome

C Symptoms are hypothermia and dehydration

D Treatment is with chemotherapy

9 Which ONE of these statements is NOT TRUE concerning chemotherapy for
acute myeloid leukaemia?

A Side-effects of treatment include increased risk of infection

B Use of growth factors such as granulocyte colony-stimulating factor (G-CSF)

can reliably prevent the development of neutropenia

C It is given as several courses of therapy which allow recovery of normal

myelopoiesis between cycles
 D Patients often need blood product support, particularly with red cell and
platelet transfusions
10 Which ONE of these is used in treatment of relapsed acute myeloid
leukaemia?

A Arsenic

B Digoxin

C Cyanide

D Atropa belladonna
11 Which ONE of these is the most accurate description of the pattern of
incidence of acute lymphoblastic leukaemia?

A Peak in first year of life, secondary rise at age 10 and then gradual decline
during adulthood

B Peak in first 3–5 years of life, less common with age but gradual rise after
age 60 years

C Peak at age 5–10 years with secondary peak at age 60 years

D Stable but high incidence in first 18 years and then gradual decline in
adulthood
12 Which ONE of these is the most likely clinical presentation of a child with
acute lymphoblastic leukaemia?

A A 6-month history of fatigue and repeated upper respiratory tract infection

B Poor appetite and abdominal pain resulting from swollen spleen

C Swollen gums in the mouth

D Recent history of bruising and tiredness

13 Which ONE of these is least likely to be considered in the differential
diagnosis of acute lymphoblastic leukaemia (ALL) in an child?

A Aplastic anaemia

B Chronic lymphocytic leukaemia

 C Neuroblastoma

D Acute myeloid leukaemia

14 Which ONE of these is NOT associated with poor clinical prognosis in
acute lymphoblastic leukaemia?

A Hyperdiploidy

B High presenting white cell count

C Philadelphia chromosome

D Presentation in infants less than 1 year old

15 Which ONE of these is NOT required for the definition of achieving a
remission during treatment for acute lymphoblastic leukaemia?

A Normal peripheral blood count

B No other symptoms or signs of the disease

C Less than 5% blasts in the bone marrow

D No leukaemia cells in bone marrow detectable by polymerase chain reaction

(PCR) analysis
16 Which ONE of these statements is most accurate concerning acute
lymphoblastic leukaemia associated with the Philadelphia chromosome?

A Tyrosine kinase inhibitors must be used on their own and usually give long-
term control

B Tyrosine kinase inhibitors have improved the prognosis

C Tyrosine kinase inhibitors prevent the need to consider allogeneic stem cell
transplantation

D Does not occur following initial therapy for chronic phase chronic myeloid
leukaemia
17 Which ONE of these is the most accurate statement concerning the cure
rate in acute lymphoblastic leukaemia?

A 65% for children and 25% for those over 70 years

 B 65% for children and <5% for those over 70 years

C 85% for children and 25% for those over 70 years

D 85% for children and <5% for those over 70 years

18 Which ONE of the following statements is NOT TRUE about acute
lymphoblastic leukaemia?

A It has a better prognosis in females than males

B It may be associated with the Philadelphia chromosome

C It causes meningeal leukaemia in 50% of cases

D It has a cure rate in children of more than 80%

 LPN

1 Which ONE of the following is a favourable feature of chronic lymphocytic

leukaemia?

A Young age

B Male sex

C Somatic mutation of immunoglobulin heavy-chain gene

D Mutation of the p53 gene

E Rapid doubling time of blood lymphocytic count

2 Which ONE of the following drugs is NOT useful in the treatment of
chronic lymphocytic leukaemia?

A Cytosine arabinoside

B Fludarabine

C Rituximab (anti-CD20)

D Chlorambucil
3 Which ONE of the following is a feature of chronic lymphocytic leukaemia?

A Herpes zoster infection

B Meningeal involvement

C Increased blasts in the bone marrow

D Bone lesions
4 Which ONE of the following is present in >50% of newly presenting cases
of chronic lymphocytic leukaemia?

A Serum paraprotein

B Autoimmune haemolytic anaemia

C CD5-positive B lymphocytes in peripheral blood

D Thrombocytopenia E Lymphadenopathy
5 Which ONE of these statements is most correct concerning the
epidemiology of B-cell chronic lymphocytic leukaemia (B-CLL)?

A It has an equivalent incidence across the world with a peak age of onset at
60–80 years

B It is more common in Caucasian populations with a bimodal peak of incidence

at 50 and 80 years

C It has an increased frequency in first-degree relatives of affected

individuals

D There is an increased incidence following radiotherapy or chemotherapy

6 Which ONE of the following statements is NOT TRUE regarding monoclonal
B-cell lymphocytosis?

A It is a precursor of B-CLL

B It is associated with immune deficiency

C It occurs in greater than 1% of the population over the age of 40 years

D It may be associated with a normal peripheral blood lymphocyte count

It is asymptomatic.
7 What is the typical phenotype of a B-CLL tumour cell?

A CD19+ CD5+

B CD19+ CD5−

C CD19− CD5+

D CD3+ CD5+
8 Which ONE of these is the most common presentation of B-CLL at the time
of INITIAL diagnosis?

A Anaemia caused by autoimmune haemolysis

B Patient is asymptomatic but has increased white cell count

C Enlarged lymph nodes

D Severe and prolonged infection

9 Which ONE of these autoimmune conditions is NOT increased in incidence in
B-CLL?

A Multiple sclerosis

B Red cell aplasia

C Autoimmune thrombocytopenia

D Autoimmune haemolysis
10 Which ONE of these statements is most accurate concerning the use of
chemotherapy for treatment of B-CLL?

A Antibodies against CD19 are useful in most cases

B Chemotherapy is given on its own and then antibodies directed against the B-
cell membrane are useful to treat relapsed disease

C Antibodies against CD20 lead to alopecia and severe pancytopenia

D It usually involves a combination of chemotherapy drugs and an antibody

against CD20
11 Which ONE of these is the most likely clinical presentation with hairy cell
leukaemia?

A A 50-year-old man with infection and abdominal swelling. Blood count shows
pancytopenia and monocytopenia

B A 70-year-old woman with anaemia. Blood count shows markedly raised white
cell count

C A 30-year-old man with anaemia and a marked thrombocytopenia. Presents

with purpura

D A 40-year-old woman with swollen lymph node in left side of neck. Increased
platelet count on blood film
12 A 67-year-old man with rheumatoid arthritis presents with neutropenia.
The blood count shows a mild lymphocytosis with an increased number of cells
which express CD3, CD16 and CD57. What is the diagnosis in this case?

A Mycosis fungoides

B Large granular lymphocytosis

C Infectious mononucleosis

D Chronic lymphocytic leukaemia

13 Which ONE of these statements is TRUE regarding adult T-cell
leukaemia-lymphoma?

A It is usually cured with CHOP chemotherapy regimen

B It is caused by infection with HIV infection

C The tumour cells have a CD4+ CD25+ phenotype

D It is usually associated with hypocalcaemia

14 Which ONE of these statements is NOT TRUE concerning ibrutinib in the
treatment of chronic lymphocytic leukaemia?

A It is an inhibitor of the Bruton tyrosine kinase (BTK) protein

B It is effective against tumours that carry a mutation in the p53 gene

C It may be associated with an increase in the lymphocyte count in peripheral

blood

D Treatment courses usually require weekly intravenous infusions

15 Which ONE gene is mutated in nearly every case of hairy cell leukaemia?

A SF3B1

B p53

C BRAF

D ATM
 MM
1 Which ONE of these is NOT associated with paraproteinaemia?

A Chronic lymphocytic leukaemia

B Lymphoplasmacytic lymphoma

C Primary amyloidosis

D Chronic myeloid leukaemia

2 Which ONE of these statements is NOT TRUE regarding multiple myeloma?

A It is relatively more common in black rather than white individuals

B The phenotype of the malignant plasma cell is that of CD38highCD138high and

CD45low

C Dysregulation of the cyclin A gene is seen in most tumours

D A central growth factor for plasma cells is interleukin-6

The cyclin D gene that is dysregulated
3 Which ONE of these is a relatively common complication in multiple
myeloma?

A Acute renal failure

B Cardiomyopathy

C Diarrhoea

D Pulmonary fibrosis
4 Which ONE of these statements is NOT TRUE regarding serum free light
chains?

A They reflect the production of immunoglobulin light chains that do not pair
with heavy chains

B An abnormal serum free light chain ratio is seen in renal failure

C They are valuable in the diagnosis of non-secretory multiple myeloma

 D They are useful in predicting which cases of monoclonal gammopathy of
undetermined significance (MGUS) may progress to multiple myeloma
Because both the kappa and lambda light chains are increased in serum and so the
ratio remains in the normal range
5 Which ONE of these clinical cases is an example of smouldering multiple
myeloma?

A Plasma cells >10% in the bone marrow, no paraprotein in blood and presence
of lytic bone lesions

B Plasma cells >10% in bone marrow, abnormal serum free light chain ratio and
hypercalcaemia

C Plasma cells <1%, paraprotein in blood and osteoporosis

D Plasma cells >10%, paraprotein 30 g/L in blood and no tissue damage

6 Which ONE of these treatment strategies is most likely to be used for a
64-year-old man who is diagnosed with multiple myeloma who is otherwise
well?

A Approximately four courses of chemotherapy followed by collection of

autologous stem cells and transplant after melphalan conditioning

B Monthly courses of oral melphalan with prednisolone until the paraprotein

level reaches a plateau, then thalidomide maintenance

C High dose chemotherapy followed by a related donor allogeneic stem cell

transplant

D Monthly courses of R-CHOP chemotherap

7 Which ONE of these is a side-effect of treatment with thalidomide?

A Hyperactivity

B Bleeding

C Myopathy

D Constipation
8 Which ONE of these statements is NOT TRUE concerning monoclonal
gammopathy of undetermined significance (MGUS)?

A Approximately 1% of cases progress to multiple myeloma each year

B It is thought to be the precursor of approximately 50% of cases of multiple

myeloma

C It is not associated with clinical symptoms

D Plasma cell numbers are usually less than 10% in the bone marrow
MGUS precedes almost all cases of myeloma

Which ONE of these statements is the best definition of amyloidoses?

A The amyloidoses are a heterogeneous group of disorders characterized by

the intracellular deposition of protein in an abnormal fibrillar form

B The amyloidoses are a heterogeneous group of disorders characterized by

the intracellular deposition of protein in an abnormal β-pleated sheet

C The amyloidoses are a heterogeneous group of disorders characterized by

the extracellular deposition of protein in an abnormal fibrillar form

D The amyloidoses are a heterogeneous group of disorders characterized by

the extracellular deposition of protein in an abnormal β-pleated sheet
10 Which ONE of these is NOT a typical clinical feature of systemic
amyloidosis?

A Macroglossia

B Peripheral neuropathy

C Cardiomyopathy

D Liver cirrhosis
 CML
1 Which ONE of these statements is TRUE concerning the translocation that
leads to the Philadelphia chromosome?

A It leads to increased expression of the c-ABL gene as it brings a strong gene

promoter close to the c-ABL gene

B It is present in around 60% of cases of CML

C It is detected on a karyotype as the t(8;21) translocation

D It leads to generation of a bcr-abl fusion protein

2 Which ONE of these statements is NOT TRUE concerning the epidemiology
of chronic myeloid leukaemia (CML)?

A It is more common in Caucasian rather than black individuals

B It can occur at any age

C The sex incidence is approximately equal

D The peak age of onset is 40–60 years

3 Which ONE of these clinical features is commonly seen in patients who
present with chronic myeloid leukaemia?

A Swollen cervical lymph nodes

B Enlarged spleen

C Bone marrow failure with reduced peripheral blood cell count

D Swelling of the gums

4 Which ONE of these is the most typical blood count in a patient presenting
with chronic myeloid leukaemia?

A Hb 3 g/dL; WBC 60 × 109/L; platelets 500 × 109/L

B Hb 9 g/dL; WBC 60 × 109/L; platelets 400 × 109/L

C Hb 6 g/dL; WBC 12 × 109/L; platelets 50 × 109/L

D Hb 16 g/dL; WBC 160 × 109/L; platelets 500 × 109/L

5 Which ONE of these statements is the most accurate definition of the
mode of action of imatinib?

A It is a specific inhibitor of the BCR-ABL fusion protein and blocks

phosphatase activity by interaction with the enzyme site

B It is a specific inhibitor of the BCR-ABL fusion protein and blocks

phosphatase activity by competing with adenosine triphosphate (ATP) binding

C It is a specific inhibitor of the BCR-ABL fusion protein and blocks tyrosine

kinase activity by interaction with the enzyme site

D It is a specific inhibitor of the BCR-ABL fusion protein and blocks tyrosine

kinase activity by competing with adenosine triphosphate (ATP) binding
Which ONE of these is considered as an optimal response to imatinib
treatment for chronic myeloid leukaemia?

A A BCR-ABL1 ratio of ≤10% after 6 months of treatment

B Failure to find any Philadelphia-positive chromosomes in bone marrow samples

after 6 months

C Failure to detect any mutations within the BCR-ABL gene after 12 months

D A complete haematological response by 6 months

7 Which ONE of these statements is NOT TRUE regarding the treatment of
chronic myeloid leukaemia with imatinib?

A Over 95% of patients tolerate the drug and go on to have long-term relapse-
free survival

B It is associated with side-effects of skin rash, fluid retention and nausea

C It is generally given at a dosage of 400 mg/day although this may be

increased

D G-CSF may be useful in the management of neutropenia

8 Which ONE of these treatments is unlikely to be considered as appropriate
treatment for a patient with failure to respond to imatinib for treatment of
chronic myeloid leukaemia?

A Dasatinib
 B Autologous stem cell transplantation

C Allogeneic stem cell transplantation

D Increase the dose of imatinib

9 Which ONE of these statements is NOT TRUE concerning juvenile chronic
myelomonocytic leukaemia?

A Clinical features include skin rashes, lymphadenopathy and

hepatosplenomegaly

B A high haemoglobin F level is a useful diagnostic feature

C The Philadelphia chromosome is present

D The blood film shows monocytosis

MPN
1 Which three genes are mutated, collectively, in over 90% of cases of
myeloproliferative disease?

A JAK2, CALR and TET2

B JAK2, CALR and MPL

C JAK2, TET2 and MPL

D CALR, TET2 and MPL

2 Which ONE of the following is NOT a cause of polycythaemia?

A Mutation of JAK-2

B Renal disease

C Congenital heart disease

D Haemoglobin abnormality

E Iron overload
3 Which ONE of the following does NOT cause a raised platelet count?

A Haemorrhage
 B Chronic myeloid leukaemia

C Mutation of JAK-2

D Pregnancy
4 Which ONE of the following is NOT a typical feature of primary
myelofibrosis?

A It causes a leuco-erythroblastic blood film

B It may be associated with a raised platelet count

C Normal serum lactate dehydrogenase level

D It may be complicated by gout

E It may cause massive splenomegaly

5 Which ONE of these statements is TRUE about pseudo (stress)
polycythaemia?

A It is caused by a raised red cell mass

B It is associated with a large spleen

C It is treated with hydroxycarbamide (hydroxyurea)

D It is most common in young male adults

6 Which ONE of the following is associated with systemic mastocytosis?

A JAK2 mutations

B Chronic myeloid leukaemia

C Urticaria pigmentosa

D Raynaud disease
7 What is the approximate frequency of the Val617Phe mutation in JAK2 in
myeloproliferative neoplasms?

A 95% in polycythaemia vera (PV) and 50% in essential thrombocythaemia (ET)

and primary myelofibrosis (PM)

B Approximately 50% in PV, ET and PM

 C 50% in PV and 25% in ET and PM

D 90% in PV, rare in ET and PM

8 Which level of haematocrit is sufficient to define an increase in the red
cell mass without the need for radioisotope studies?

A 0.5

B 0.55

C 0.6

D 0.65
9 What is the normal range of red cell volume for a man?

A 20–25 ml/L

B 25–35 ml/L

C 20–25 ml/kg

D 25–35 ml/kg
10 Which ONE of these clinical features is often seen in patients with
polycythaemia vera?

A Nocturnal cough

B Increased incidence of gallstones

C Pruritus (itch) after a bath

D Lymphadenopathy
11 Which ONE of these is NOT a feature of polycythaemia vera?

A Splenomegaly

B Neutrophil leucocytosis

C High serum erythropoietin

D Thrombocytosis
12 What is the approximate risk of progression to myelofibrosis or acute
myeloid leukaemia in patients with polycythaemia vera?
 A 5% and 30%, respectively

B 10% and 10%, respectively

C 20% and 20%, respectively

D 30% and 5%, respectively

13 Mutation in which ONE of these genes is NOT associated with congenital
polycythaemia?

A EPO

B VHL

C PHD2

D HIF2A
14 Which ONE of these is the best definition of erythromelalgia, a clinical
symptom of essential thrombocythaemia?

A Headache, most common after exercise

B A burning sensation felt in the hands or feet

C Red facies seen most commonly in the morning

D Raised red patches on the fingers and toes

15 Which ONE of these statements regarding primary myelofibrosis is TRUE?

A Stem cell transplantation is not a curative option

B A leuco-erythroblastic blood film is a characteristic feature

C The spleen is rarely enlarged

D Uric acid levels are typically low

16 Which ONE of the following is true regarding JAK2 inhibitors?

A They are effective only in patients with JAK2 mutated myeloproliferative

disease

B They are effective at reducing spleen size in primary myelofibrosis

 C They are useful in treatment of myelodysplasia

D They are used in the treatment of pseudo-polycythaemia

MDS
1 Which ONE of these is the most common finding in myelodysplastic
syndromes (MDS)?

A Hypocellular bone marrow and reduced blood cell counts

B Hypocellular bone marrow and increased blood cell counts

C Hypercellular bone marrow and reduced blood cell counts

D Hypercellular bone marrow and increased blood cell counts

2 Which ONE of these is NOT associated with dysplastic changes in the bone
marrow?

A Haemolytic anaemia

B Excess alcohol intake

C Treatment with G-CSF

D Recovery from cytotoxic chemotherapy

3 Which ONE of these is NOT a typical feature of dysplastic peripheral
blood cells in myelodysplastic syndromes?

A Increased haemoglobin concentration in red cells

B Macrocytic red cells

C Hypogranular neutrophils

D Bilobed nucleus in neutrophils

4 What percentage of blast cells in the bone marrow would indicate a
diagnosis of acute myeloid leukaemia rather than myelodysplasia?

A 5%

B 10%

C 20%
 D 30%
5 Which ONE of these is NOT a diagnostic subtype of myelodysplasia?

A Refractory anaemia with ring sideroblasts

B Refractory anaemia with excess blasts

C Refractory cytopenia with unilineage dysplasia

D X-linked sideroblastic anaemia

6 Which ONE of these is the most likely clinical picture in a patient with
myelodysplastic syndrome associated with isolated del(5q)?

A A man with Hb 13 g/dL and a platelet count of 600 × 109/L

B A woman with Hb 10 g/dL and a platelet count of 600 × 109/L

C A woman with Hb 13 g/dL and a platelet count of 100 × 109/L

7 Which ONE of these is most likely to be used as the treatment for a 75-
year-old patient with refractory anaemia and haemoglobin 90 g/L?

A Azacytidine

B Stem cell transplantation

C Lenalidomide

D Trial of erythropoietin
8 Which ONE of these is the most powerful predictive factor in patients with
myelodysplastic syndrome?

A Serum β2-microglobulin

B Gender

C The number of blasts in the bone marrow

D The level of anaemia

9 In which patients with myelodysplastic syndrome is ciclosporin most likely to
be effective?

A Low risk disease with hypocellular bone marrow

 B Low risk disease with hypercellular bone marrow

C High risk disease with hypocellular bone marrow

D High risk disease with hypercellular bone marrow

10 Which ONE of these statements is the best definition of chronic
myelomonocytic leukaemia?

A Persistent monocytosis of >10.0 × 109/L with blasts <20% in the marrow,

dysplasia in other lineages and negative for BCR-ABL1

B Persistent monocytosis of >1.0 × 109/L with blasts >20% in the marrow,

dysplasia in other lineages and negative for BCR-ABL1

C Persistent monocytosis of >1.0 × 109/L with blasts <20% in the marrow,

dysplasia in other lineages and negative for BCR-ABL1

D Persistent monocytosis of >1.0 × 109/L with blasts <20% in the marrow,

dysplasia in other lineages and positive for BCR-ABL
11 Which ONE of these statements is TRUE regarding the use of
erythropoietin (EPO) in the management of patients with myelodysplasia?

A It should be used to get the haemoglobin concentration as high as possible

B It is associated with a risk of bleeding

C It needs to be given every week as an infusion on a hospital ward

D It is likely to be more effective in patients with a low endogenous

erythropoietin level
12 Genetic analysis shows that a patient with MDS has a mutation in
the SF3B1 gene. Which ONE subtype of MDS is the most likely diagnosis?

A Refractory anaemia with ring sideroblasts

B Chronic myelo-monocytic leukaemia

C Juvenile myelomonocytic leukaemia

D Atypical chronic myeloid leukaemia (BCR-ABL1 negative)