Myelodysplastic Syndrome and Myeloproliferative Neoplasms Flashcards

1
Q

MDS presents with ________.

A

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

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2
Q

Chromatin will often look _______ in dysplastic myeloid cells.

A

“moth-eaten”

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3
Q

What are the three classifications of myeloproliferative disorders?

A

low grade MDS (20% myeloblasts)

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4
Q

Myeloproliferative neoplasms are characterized by __________.

A

an increase in one line of functional hematopoietic cells

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5
Q

CML is defined by _________.

A

the presence of the BCR-ABL1 translocation

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6
Q

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

A

AML

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7
Q

Polycythemia vera is characterized by ________.

A

erythrocytosis, increases in other myeloid cells

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8
Q

Other than PV, what patients might have erythrocytosis?

A

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

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9
Q

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

A

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

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10
Q

What karyotypic findings are suggestive of MDS?

A

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

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11
Q

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

A

toxin exposure, anemia, and nutrient deficiency

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12
Q

What are typical prognoses for MDS?

A

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

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13
Q

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

A

fibrotic scarring, ineffective hematopoiesis, or transition to AML

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14
Q

Which disorders have hepatosplenomegaly?

A

MPNs

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15
Q

What are the four main types of MPNs?

A

Polycythemia vera, essential thrombocythemia, CML, and primary myelofibrosis

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16
Q

What is the typical presentation age for CML?

A

40 - 50

17
Q

What was the second-generation Gleevec called?

A

Dasatinib

18
Q

Polycthemia vera should be considered in those with _________.

A

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

19
Q

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

A

paresthesias

20
Q

PV has a ________ prognosis.

A

good (10-20 year survival)

21
Q

Primary myelofibrosis is like PV but without ________.

A

erythrocytosis

22
Q

Essential thrombocythemia and primary myelofibrosis both have ______ mutations.

A

JAK2