Leukemias Flashcards
Hallmark of CML
Philadelphia chromosome
9;22 translocation
Bcr-alb
Leads to increased cell division and inhibition of apoptosis
JAK2 negative!
High WBC and platelets
Polycythemia vera definition
Abnormal clone of hematopoietic stem cells are increasingly sensitive to growth factors
High RBC, WBC, Platelets
JAK2 positive
Red cells are produced in excessive amounts without hypoxia or EPO stimulation
Essential thrombocythemia
Similar to polycythemia vera, but specific for megakaryocytes- “platelet cancer”
High platelets (only) millions! JAK2 positive
Myelofibrosis
Fibrotic obliteration of bone marrow. Teardrop cells
Low RBC
JAK2 positive
First line treatment for CML
Imatibib, direct tyrosine kinase inhibitor
How does a polycythemia vera patient present?
Markedly elevated HCT**** Splenomegaly Sometimes elevated platelets and white cells Thrombosis Plethora of redness, fullness of face
Dx: rule out CO poisoning, check EPO levels, check JAK
Polycythemia treatment
Hydroxyurea
4 types of myeloproliferative neoplasms
1) myelofibrosis
2. Polycythemia vera
3) essential thrombocytosis
4. Chronic myelogenous leukemia
Which MPN is most common?
Which 2 MPNs have near normal life expectancy if treated?
Which is deadliest?
- PV
- CML ET
- MF
Which MPN has the worst survival without treatment?
CML
MPNs common clinical elements
- Splenomegaly
- BM hypercellularity
- Increased risk of thrombosis or bleeds
- B symptoms: weight loss, drenching night sweats, fever, fatigue
Which is the worst phase of CML?
Blast phase, prognosis 3-6 months
What does CML CBC show? BM?
Elevated WBC with left myeloid shift
Low Hg and HCT because there’s no room in BM
BM shows hypercellularity (should be 100- age)
Which MPN causes you to itch after a hot shower?
Polycythemia vera
Polycythemia vera treatment
Hydroxyurea if over 60, low dose aspirin if indication, phlebotomy to maintain HCT
