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
cytogenetics PV
JAK-2
Normal JAK-STAT pathway
cell signaling pathway
important in many different cell types
JAK-STAT in PV
Mutated JAK-2: activity increased in PV
Cells grow on their own
tx PV
Phlebotomy
Maybe myelosuppressive drugs
prognosis PV
Median survival: 9-14 years
Death from thrombosis or hemorrhage
Leukemic transformation in some patients
Essential thrombocythemia
things you must know
Very high platelet count in blood
Can occur in young women
Diagnosis of exclusion
Thrombosis and hemorrhage
Dx criteria for ET
Platelet count >600,000
Hgb
signs and sxs ET
Bleeding
Thrombosis
Purpura, bruising
Pallor, tachycardia
Biggish spleen
tx ET
Platelet pheresis
Maybe myelosuppressive drugs
Aspirin
Prognosis ET
Median survival: 5-8 years
Death from thrombosis or hemorrhage
Leukemic transformation in some patients
Chronic myelofibrosis
THINGS you must know
Panmyelosis then marrow fibrosis
Extramedullary hematopoiesis
Teardrop red cells
Clinical findings MF
Symptoms
Left upper quadrant fullness
Weakness, fatigue, palpitations
Signs
Huge spleen
Pallor, tachycardia
tx MF
Supportive
Maybe myelosuppressive drugs (early on)
prognosis MF
Median survival: 3-5 years
Death from marrow failure
Leukemic transformation in some patients
