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
20% or more blasts is the definition of
Acute Leukemia
The presence of Auer Rods seen in
Acute myeloid Leukemia
Auer rods: abnormal crystallized azurophilic granules, particularly in promyelocytic leukemia
Terminal deoxynucleotidyl transferase is positive or negative in Acute Myeloid Leukemias?
negative because TdT is used for gene rearrangement in lymphoid precursor cells and isn’t as present in myeloid lineage
CD markers for AML?
CD13,33,34,117; Sudan Black +, Myeloperoxidase +
TdT negative
Genetic Aberrations in AML w/ favorable prognosis
t(15;17) Acute Promyleocytic Leukemia (RAR-a defect)
t(8;21) Favorable prognosis
inv(16) or t(16;16) Automyelomonocytic Leukemia w/abnml eosinophils favorable prognosis
Genetic Aberrations in AML w/ poor prognosis
5q-, 7q-
Also: greater than 60yo, leukocytosis >100,000
