6.4 - Myeloproliferative disorders Flashcards
how are myeloproliferative disorders classified?
based on dominant myeloid cell produced
what are the myeloproliferative disorders?
- CML
- polycythemia vera
- essential thrombocytopenia
- myelofibrosis
what cell type is classically elevated in CML?
basophil
CML is driven by what translocation? what does it generate?
- t(9,22)
- generates BCR-ABL fusion protein with increased tyrosine kinase activity
what is the first line treatment for CML?
imatinib
what physical sign suggests progression to accelerated phase of disease in CML?
splenomegaly
CML can transform to what other cancers?
- AML
- ALL
how is CML differentiated from leukemoid reaction?
CML shows:
- negative LAP stain
- basophilia
- t(9,22) translocation
PV is associated with what mutation?
JAK2
what are the two treatments for PV?
- phlebotomy (first line)
- hydroxyurea
how is PV differentiated from reactive polycythemia?
PV shows:
- EPO is decreased
- SaO2 is normal
how is PV differentiated from reactive polycythemia due to ECTOPIC EPO?
polycythemia due to ECTOPIC EPO shows:
- EPO is elevated
- SaO2 is normal
essential thrombocytopenia is associated with what mutations?
JAK2 kinase
myelofibrosis is associated with what mutation?
JAK2 kinase
in myelofibrosis, megakaryocytes produce excess ___________, leading to ____________
- PDGF
- marrow fibrosis
