6.4 - Myeloproliferative disorders Flashcards Preview

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Flashcards in 6.4 - Myeloproliferative disorders Deck (16)
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1
Q

how are myeloproliferative disorders classified?

A

based on dominant myeloid cell produced

2
Q

what are the myeloproliferative disorders?

A
  • CML
  • polycythemia vera
  • essential thrombocytopenia
  • myelofibrosis
3
Q

what cell type is classically elevated in CML?

A

basophil

4
Q

CML is driven by what translocation? what does it generate?

A
  • t(9,22)

- generates BCR-ABL fusion protein with increased tyrosine kinase activity

5
Q

what is the first line treatment for CML?

A

imatinib

6
Q

what physical sign suggests progression to accelerated phase of disease in CML?

A

splenomegaly

7
Q

CML can transform to what other cancers?

A
  • AML

- ALL

8
Q

how is CML differentiated from leukemoid reaction?

A

CML shows:

  • negative LAP stain
  • basophilia
  • t(9,22) translocation
9
Q

PV is associated with what mutation?

A

JAK2

10
Q

what are the two treatments for PV?

A
  • phlebotomy (first line)

- hydroxyurea

11
Q

how is PV differentiated from reactive polycythemia?

A

PV shows:

  • EPO is decreased
  • SaO2 is normal
12
Q

how is PV differentiated from reactive polycythemia due to ECTOPIC EPO?

A

polycythemia due to ECTOPIC EPO shows:

  • EPO is elevated
  • SaO2 is normal
13
Q

essential thrombocytopenia is associated with what mutations?

A

JAK2 kinase

14
Q

myelofibrosis is associated with what mutation?

A

JAK2 kinase

15
Q

in myelofibrosis, megakaryocytes produce excess ___________, leading to ____________

A
  • PDGF

- marrow fibrosis

16
Q

what is seen on blood smear in myelofibrosis?

A
  • leukoerythroblastic
  • tear drop RBCs (dacrocytes)
  • nucleated RBCs
  • immature granulocytes

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