MPNs

Question 1

are clonal hematopoietic disorders caused by genetic mutations in the hematopoietic stem cells (HSCs)

Answer 1

myeloproliferative neoplasm

Question 2

what are the 4 predominant disorders of MPNs according to the WHO

Answer 2

chronic myeloid leukemia (CML)
polycythemia vera (PV)
essential thrombocytopenia (ET)
primary myelofibrosis (PMF)

Question 3

ET, PV, and PMF are related based on the presence of

Answer 3

Janus Kinase 2 (JAK2)

Question 4

in ET, PV, and PMF what is absent

Answer 4

philadelphia chromosome or BCR ABL1 fusion gene

Question 5

a MPN arising from a single genetic translocation in a pluripotential HSCs producing a clonal overproduction of the myeloid cell line

Answer 5

chronic myeloid leukemia

Question 6

the chromosome associated for CML

Answer 6

t(9:22)

Question 7

the detection of what fusion gene is useful for the diagnosis of CML

Answer 7

BCR-ABL1 fusion gene

Question 8

what enzyme is useful for the preliminary differentiation of CML and Leukemoid Reaction

Answer 8

Leukocyte Alkaline Phosphatase (LAP)

Question 9

it is an enzyme found on the secondary granules

Answer 9

LAP

Question 10

treatment for CML

Answer 10

imatinib mesylate

Question 11

is a neoplastic clonal MPN that commonly manifests with panmyelosis in the BM

Answer 11

polycythemia vera

Question 12

mutation in PV

Answer 12

JAK2 V617F

Question 13

JAK2 V617f exon F is found on what chromosome band

Answer 13

9p24

Question 14

diagnosis of PV

Answer 14

elevated HGB, HCT
BM showing hypercellularity (panmyelosis)
identification of JAK2 V617F

Question 15

treatment for PV

Answer 15

therapeutic phlebotomy
myelosuppressive therapy
targeted molecular therapy

Question 16

an alkylating agent for PV

Answer 16

hydroxyurea

Question 17

a clonal MPN with an increased megakaryopoiesis and thrombocytosis

Answer 17

essential thrombocythemia

Question 18

reference range of WHO to consider as ET

Answer 18

greater than 400 x 10^9/L