Myeloproliferative Neoplasms Flashcards

1
Q

All MPNs can present with

A

Thromboses
Splenomegaly
Systemic symptoms

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2
Q

CML

A

translocation 9;22
Philadelphia chromosome
BCR-ABL

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3
Q

Accelerated CML/ Blast crisis

A

AML

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4
Q

Characteristics of CML

A

splenomegaly

elevated leukocyte count

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5
Q

Granulocyte=

A

WBC (neutrophil, eosinophil, basophil, monocyte)

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6
Q

Common myeloid progenitor makes

A

Erythrocyte, Mast cells, megakaryocyte and myeloblast

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7
Q

Myeloblasts make

A

neutrophil, eosinophil, basophil, monocyte

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8
Q

Peripheral smear of CML patient

A

granulocytes in all phases of maturation

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9
Q

When blasts represent 10-20% of leukocytes

A

Accelerated phase of CML

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10
Q

When blasts represent >20% of leukocytes

A

Blast phase of CML

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11
Q

Treatment for CML

A

Hydroxyurea
Tyrosine Kinase inhibitor
HSCT

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12
Q

Tyrosine Kinase inhibitors

A

Imatinib, Mesylate, Dasatinib, Nilotibin

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13
Q

Side effect of tyrosine kinase inhibitors

A

Qtc prolongation

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14
Q

Most common MPN

A

Essential Thrombocythemia

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15
Q

In Essential Thrombocythemia there is a marked increase in

A

Megakaryocytes and Platelets

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16
Q

Symptoms of Essential Thrombocythemia

A

erythromelalgia (red, painful hands and feet)
Livedo reticularis
Thrombosis

17
Q

Mutation in ET

A

Jak2

18
Q

Treatment for low risk ET

A

Aspirin

19
Q

Treatment for high risk ET patients

A

Hydroxyurea + Aspirin

20
Q

Emergent treatment for ET

A

Platelet pheresis

21
Q

Hgb >16.5 in men; >16 in women

A

Polycythemia Vera

22
Q

Mutation in PCV

A

Jak2

23
Q

Microscopic hematuria + elevated hemoglobin and erythrocyte

A

erythropoitin hypernephroma

24
Q

Treatment for PCV

A

Therapeutic phlebotomy. Goal Hct 45%

Hydroxyurea, Aspirin (low dose)

25
Q

Hydroxyurea and Aspirin can treat

A

PCV
ET
CML (just hydroxyurea-palliative)

26
Q

Budd chiari or portal vein thrombosis

A

think of PV

27
Q

Primary myelofibrosis treatment

A

Supportive
HSCT for ppl <60
Hydroxyurea, ruxxolitinib (Jak2 inhibitor) for symptoms