CML Flashcards
Adults or kids or both?
Adults ONLY
Four types of Chronic myeloproliferative disorders?
Chronic myeloid leukemia (neuts)
Polycythemia vera (RBC’s)
Essential thrombocythemia (platelets)
Myelofibrosis (little bit of everything)
Characteristics common to all four disorders?
Leukocytosis w/ LEFT SHIFT
Hypercellular marrow —>
Big spleen
long clinical course
only in adults
may evolve into acute leukemia
mutated tyrosine kinase (an activating mutation)
Things you must know about CML:
- Neutrophilic leukocytosis
- Basophilia
- PHILADELPHIA chromosome
- three phases
What does the Hgb look like in CML?
LOW
What’s LAP and what does it mean?
Leukocyte Alkaline Phosphate
**decreased in CML
**INcreased in benign leukocytosis
What are the platelets gonna look like in CML?
Increased at first (lots of malignant megakaryocytes too) then decrease later when neuts take over
Three phases of CML:
Chronic phase
Accelerated phase
Bast crisis
Criteria for hematologic remission in CML:
no splenomegally
WBC
Criteria for cytogenic remission?
no metaphases with t(9:22)
Molecular remission criteria of CML?
No BCR/ABL transcripts by PCR
4 things you must know about Polycythemia vera?
HIGH RBC (makes blood sludgy)
different from secondary polycythemia
thrombosis and hemorrhage
Jak-2 mutation
Difference between primary and secondary polycythemia?
primary– intrinsic myeloid cell prob
secondary– increased erythropoietin (ie. paraneoplastic)
Clinical sx of PV?
HA, pruritis, dizziness
-thrombosis, infarction
physical exam findings:
hepatosplenomegally
“Plethora” – old school term for flushing face/cheeks
Whats the issue with the JAK-2 mutation in PV?
Increased activity–> cells grow on their own without external stimulus
Tx for PV:
Phlebotomy
maybe myelosupressive drugs