CML Flashcards
Chronic myeloproliferative disorders: Malignant proliferation of what
Malignant proliferation of myeloid cells (not blasts, but maturing cells) in blood, bone marrow
Chronic myeloproliferative disorders (4)
CML
PV
ET
MF
CMD only happen in what people
adults
course of CMD
long
What is proliferating most in CML, PV, ET, FM
CML - neutrophils
PV - red cells
ET - platelets
MF - everything!
features common to all 4 disorders
Occur only in adults Long clinical course INC WBC with left shift Hypercellular marrow Big spleen May evolve into acute leukemia Mutated tyrosine kinases
CML things you must know
Neutrophilic leukocytosis
Basophilia
Philadelphia chromosome
Three phases
if see basophilia, think
CML!
philadelphia chromosome –>
CML!
Lab findings in CML
INC WBC neutrophilia with left shift basophilia DEC hgb INC platelet count DEC LAP
why dec LAP in CML
LAP is an enzyme in neutrophils, but in CML they don’t make this enzyme so it goes down
Leukocyte alkaline phosphatase
philadelphia chromosome
Normally: 9 has ABL oncogene, 22 has BCR locus
In CML: translocation of ABL onto 22 –> BCR-ABL hybrid gene –> tyrosine kinase
sxs CML
Slow onset
Fever, fatigue, night sweats
Abdominal fullness
signs CML
Big spleen
Big liver
Phases of CML
Chronic
Accelerated phase
Blast crisis
Chronic phase CML
Stable counts
Easily controlled
3-4 years (untreated) –> accelerated phase or blast crisis (50/50)
Accelerated phase CML
Unstable counts
Blast crisis within 6 – 12 months
Blast crisis CML
Acute leukemia
High mortality
Hematologic remission
No splenomegaly
WBC
Cytogenic remission
No metaphases with t(9;22)
Molecular remission
No BCR/ABL transcripts by PCR
Polycythemia vera things you must know
High RBC (makes blood sludgy)
Different from secondary polycythemia
Thrombosis and hemorrhage
Jak-2 mutation
sxs PV
headache, pruritis, dizziness
thrombosis, infarction
signs PV
big spleen, liver, plethora
