Chapter 15 Myeloproliferative neoplasms

You have answered 0 questions correctly. You have scored 0%.

15.1 Which ONE of the following genes is most commonly mutated in polycythaemia vera?

A JAK2

B CALR

C MPL

D TET2

15.2 A 28-year-old competitive bicyclist is found to have a haemoglobin of 202 g/L. The rest of
the full blood count is normal. At age 17 his haemoglobin was 160 g/L. The spleen is non-
palpable. Serum erythropoietin level is < 5 U/L. A myeloid gene mutation panel that
includes JAK2 is negative. His blood pressure is 155/95 mmHg. Which ONE of the
following is most likely?

A Congenital variant in VHL

B Renal tumour producing erythropoietin

C Endurance training at altitude

D Polycythaemia rubra vera

E Surreptitious use of a performance-enhancing drug that is not detectable with the serum erythropoietin
assay

15.3 Which ONE of the following tests is most useful as a test for a reactive (non-neoplastic)
cause of a raised platelet count?

A C-reactive protein

B Serum thrombopoietin

C Anti-nuclear antibody

D Serum tryptase

15.4 A 73-year-old man who has neglected his health presents to his general practitioner with
15 kg involuntary weight loss and drenching night sweats. On examination he has
generalized pallor, cachexia, massive splenomegaly and ascites. A full blood count
shows microcytic anaemia. Serum ferritin is 690 ug/L. Which ONE of the following
diagnoses is most likely?

A Hepatic cirrhosis due to haemochromatosis

B Mast cell disease

C Primary myelofibrosis

D Vitamin D deficiency
:
 15.5 Which ONE of these statements is TRUE about pseudo (stress) polycythaemia?

A Red cell mass is usually elevated

B Splenomegaly is common

C Hydroxycarbamide (hydroxyurea) is usually required to control the blood count

D Plasma volume is typically elevated

E It is most common in male smokers

15.6 A 35-year-old woman is diagnosed with systemic mastocytosis after presenting with
bone pain, ascites and urticaria. Which ONE of the following genes is most likely to be
mutated?

A JAK2

B ABL1

C KIT

D STAT3

15.7 Which ONE of the following 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

15.8 Which ONE of these clinical features is most often seen in patients with polycythaemia
vera?

A Nocturnal cough

B Increased incidence of gallstones

C Pruritus (itch) after a warm bath

D Lymphadenopathy

15.9 A 59-year-old man is diagnosed with polycythaemia vera. Which ONE of the following
findings is LEAST likely to be seen in this patient?

A Splenomegaly

B Neutrophil leucocytosis

C High serum erythropoietin

D Thrombocytosis
:
 A 69-year-old man was diagnosed with polycythaemia vera 10 years ago and has been
15.10 maintained on a program of phlebotomy, aspirin and hydroxycarbamide. His spleen has
enlarged markedly since his last clinic visit 3 months ago, and he now has a
haemoglobin of 84 g/L. His platelet count, previously normal, has dropped to 75 × 109/L;
his white count is unchanged. A few nucleated red blood cells are noted on the blood
film. Which ONE of the following is the most likely diagnosis?

A Post-polycythaemic myelofibrosis

B Acute myeloid leukaemia

C Systemic mastocytosis

D Non-Hodgkin lymphoma

E Hydroxycarbamide toxicity

15.11 Mutation in which ONE of the following genes is NOT associated with congenital
polycythaemia?

A EPO

B VHL

C PHD2

D HIF2A

15.12 A 64-year-old woman develops a femoral vein thrombosis and a full blood count shows a
platelet count of 740 × 109/L. Additional evaluation confirms a diagnosis of essential
thrombocythaemia. Which ONE of the following is the most appropriate management for
this patient?

A Anticoagulation and phlebotomy

B Anticoagulation and hydroxycarbamide (hydroxyurea)

C Serial platelet pheresis

D Chlorambucil and aspirin

15.13 A 58-year-old man is diagnosed with primary myelofibrosis after presenting with fatigue
and being found to have splenomegaly. He has transfusion-dependent anaemia, 3%
circulating blasts and ASXL1, MPL and SRSF2 mutations. Marrow is hypercellular for
age, with increased fibrosis and < 5% blasts. He does not tolerate ruxolitinib due to
emergence of severe thrombocytopenia. An HLA-matched donor is available and his
health is good otherwise. Which ONE of the following is the most appropriate clinical
management of this patient? (P)

A Allogeneic stem cell transplantation

B Aspirin and observation

C Androgens and hydroxycarbamide (hydroxyurea)

D Induction chemotherapy

15.14 A 66-year-old man is diagnosed with primary myelofibrosis after presenting with
anaemia, splenomegaly and constitutional symptoms. His blood shows MPL W515L
:
 mutation; JAK2 is wild type. Marrow is hypercellular for age with megakaryocyte
clustering, and reticulin and collagen fibrosis. Which ONE of the following is TRUE about
the use of the JAK inhibitor ruxolitinib in this patient? (P)

A Ruxolitinib would not be expected to be effective in this patient since JAK2 is wild type

B Ruxolitinib is likely to improve the splenomegaly in this patient but not the anaemia

C The patient should be counselled that ruxolitinib increases the risk of secondary leukaemia

D Ruxolitinib would be expected to reverse the marrow fibrosis in the majority of patients

Submit Reset
: