Myeloid Neoplasms: Myeloproliferative & AML Flashcards
20% of Leukemias are chronic myeloid leukemia and presents w/ marked _____cytosis (2x), massive splenomegaly from extramedullary hematopoiesis, and _______philia
Granulocytosis and Leukocytosis
Basophilia
Chronic Myeloid Leukemia translocation
BCR:Abl fusion on philadelphia chromosome t(9;22) (also seen in acute lymphoblastic leukemia w/ intermediated prognosis
Also (q34;q11)
t(9;22) BCR: Abl creates what fusion protein
P210
Phases of CML
Chronic Phase (2-8 years) Accelerated Phase (10-19% blasts) Blast Crisis (70% Myeloid AML, 30% ALL)
What does Imatinib treat to downregulate the tyrosine kinase activity in Chronic Myeloid Leukemia
Abl binding domain
The JAK2 mutation in polycythemia vera causes what to be phosphorylated?
STAT5
Treatment of polycythemia vera can include a tyrosine kinase inhibitor called
Ruxolitinib
Phases of Polycythemia vera
Proliferatie Phase
Spent Phase
Post Polycythemia Myelofibrosis
AML
Primary myelofibrosis mutations
JAK2, Calreticulin
Survival of primary myelofibrosis?
3-5 years
Characteristic peripheral blood smear of Primary Myelofibrosis
Tear drop shaped RBCs from passing through fibrotic sinusoids (anisopoikilocytosis)
5-20% of those w/ Primary myelofibrosis progress to
AML
Essential thrombocytosis demonstrates proliferation of
megakaryoctyes
Essential thrombocytosis is a proliferation of megakaryocytes in the bone marrow and so will show in increase of what lab values
platelet numbers, platelets will be abnormal
Sheets of megakaryocytes seen in essential thrombocytosis, this poses a risk for
thrombosis
