Myelodysplastic Syndrome and Myeloproliferative Neoplasms

Question 1

MDS presents with ________.

Answer 1

ineffective hematopoiesis due to dysplastic growth of a clone; persistent peripheral cytopenia of multiple cell lineages; median age of 70

Question 2

Chromatin will often look _______ in dysplastic myeloid cells.

Answer 2

“moth-eaten”

Question 3

What are the three classifications of myeloproliferative disorders?

Answer 3

low grade MDS (20% myeloblasts)

Question 4

Myeloproliferative neoplasms are characterized by __________.

Answer 4

an increase in one line of functional hematopoietic cells

Question 5

CML is defined by _________.

Answer 5

the presence of the BCR-ABL1 translocation

Question 6

When CML enters the “blast phase,” it is then considered to be _______.

Answer 6

AML

Question 7

Polycythemia vera is characterized by ________.

Answer 7

erythrocytosis, increases in other myeloid cells

Question 8

Other than PV, what patients might have erythrocytosis?

Answer 8

Smokers, those who live at high altitude, those with high-affinity hemoglobin

Question 9

Under morphologic examination, what should an MDS patient’s blood demonstrate?

Answer 9

Erythrocyte precursors with abnormal nuclei and ring sideroblasts; granulocyte precursors with hypogranulation

Question 10

What karyotypic findings are suggestive of MDS?

Answer 10

Monosomy of 5 or 7; q deletions of 5 or 7; trisomy 8

Question 11

Prior to diagnosing someone with MDS, you should exclude cytopenias from ______.

Answer 11

toxin exposure, anemia, and nutrient deficiency

Question 12

What are typical prognoses for MDS?

Answer 12

Low grade has a median survival of either over five years (for one unilineage) or 2.5 years, and high grade has a survival 16 months or 9 months

Question 13

Those with myeloproliferative disorders have bone marrow dysfunction such as ________.

Answer 13

fibrotic scarring, ineffective hematopoiesis, or transition to AML

Question 14

Which disorders have hepatosplenomegaly?

Answer 14

MPNs

Question 15

What are the four main types of MPNs?

Answer 15

Polycythemia vera, essential thrombocythemia, CML, and primary myelofibrosis

Question 16

What is the typical presentation age for CML?

Answer 16

40 - 50

Question 17

What was the second-generation Gleevec called?

Answer 17

Dasatinib

Question 18

Polycthemia vera should be considered in those with _________.

Answer 18

emboli in the splenic vein, mesenteric vein, or portal vein

Question 19

“Pins-and-needles” are medically referred to as ________.

Answer 19

paresthesias

Question 20

PV has a ________ prognosis.

Answer 20

good (10-20 year survival)

Question 21

Primary myelofibrosis is like PV but without ________.

Answer 21

erythrocytosis

Question 22

Essential thrombocythemia and primary myelofibrosis both have ______ mutations.

Answer 22

JAK2