Ph-negative myeloproliferative neoplasms

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The presence of a JAK2 V617F mutation always indicates an overt myeloproliferative neoplasm. *

1 point

True

False

If a JAK2 V617F mutation is found in someone without an overt myeloproliferative neoplasm, can it be ignored? *

0 points

Yes

Which is the most common mutation in polycythemia vera? *

1 point

CALR

JAK2 V617F

JAK2 exon 12

MPL

BCR-ABL

Which mutation carries the worst prognosis in primary myelofibrosis? *

1 point

CALR (type 1)

JAK2

MPL

Which of these is a JAK2 inhibitor that can be used in the treatment of myelofibrosis and/or polycythemia vera? *

1 point

Imatinib

Nilotinib

Ruxolitinib

Dasatinib

A patient with suspected essential thrombocythemia is negative for JAK2 V617F mutation. Which is the next most common mutation? *

1 point

BCR-ABL1

JAK2 exon 12

MPL

NPM1

CALR

MPL encodes which receptor protein? *

1 point

Transforming growth factor

Angiopoietin

Stem cell factor

Thrombopoietin

In which condition are CALR mutations LEAST likely to be found? *

1 point

Essential thrombocythaemia

Primary myelofibrosis

Polycythemia vera

MDS/MPN with ring sideroblasts and thrombocytosis

Once a reactive state has been excluded, approximately what percentage of suspected primary myelofibrosis cases are triple-negative for JAK2, CALR, or MPL mutations? *

1 point

<1%

12%

57%

98%

When a CALR mutation has been found in a patient with essential thrombocythaemia, the case should also be tested for a MPL mutation as the presence of both confers a worse prognosis. *

1 point

True

False

Which of the following are NOT known to activate JAK2? *

1 point

GM-CSF

Erythropoietin

STAT3

IL-3

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