BI235 Myeloid Neoplasms Flashcards
What kind of cells are increased in chronic myeloid leukemia ?
increased numbers of WBCs - neutrophils, monocytes, basophils, eosinophils
what kind of cells are increased in essential thrombocytosis?
platelets
what kind of cells are increased in polycythemia vera
red blood cells
what do blast cells on blood smear suggest the diagnosis to be?
Either acute myeloid leukemia or acute lymphoblastic leukemia
What characterizes CML (Chronic Myeloid Leukemia)?
- it is a clonal stem cell disorder resulting in increased numbers of normal myeloid cells
Describe the mutation that causes CML.
- translocation btwn chromosomes 9 and 22
- makes new BCR-ABL protein
- this causes increased production of new myeloid cells
How does the BCR-ABL protein cause manifestations of diseases in CML?
This particular protein causes the bone marrow to make blood cells without feedback
what are the symptoms of CML?
1) can be asymptomatic
2) constitutional symptoms
3) splenomegaly
4) signs of cytopenia (fatigue, bleeding/bruising)
How can we confirm the diagnosis of CML?
1) by demonstrating the philadelphia chromosome
2) finding the BCR-ABL gene product through PCR testing
What is the treatment for CML?
tyrosine kinase inhibitors
what is an example of a tyrosine kinase inhibitor?
Imatinib
what is the mechanism of action of Tyrosine kinase inhibitors on the BCR-ABL protein?
1) TKI binds to the ATP binding site
2) prevents protein from functioning
3) results in remission of disease phenotype
What is polycythemia?
increased number of RBCs and usually increased Hb
What are the 3 types of polycythemia?
1) primary polycythemia - Jak2 mutation
2) secondary polycythemia - due to increase in erythropoietin levels
3) relative polycythemia - normal RBC but concentration in the blood is high due to low plasma volume
What is the most common secondary cause of increased RBCs?
chronic hypoxia from chronic lung disease
