Myeloproliferative Neoplasms Flashcards
All MPNs can present with
Thromboses
Splenomegaly
Systemic symptoms
CML
translocation 9;22
Philadelphia chromosome
BCR-ABL
Accelerated CML/ Blast crisis
AML
Characteristics of CML
splenomegaly
elevated leukocyte count
Granulocyte=
WBC (neutrophil, eosinophil, basophil, monocyte)
Common myeloid progenitor makes
Erythrocyte, Mast cells, megakaryocyte and myeloblast
Myeloblasts make
neutrophil, eosinophil, basophil, monocyte
Peripheral smear of CML patient
granulocytes in all phases of maturation
When blasts represent 10-20% of leukocytes
Accelerated phase of CML
When blasts represent >20% of leukocytes
Blast phase of CML
Treatment for CML
Hydroxyurea
Tyrosine Kinase inhibitor
HSCT
Tyrosine Kinase inhibitors
Imatinib, Mesylate, Dasatinib, Nilotibin
Side effect of tyrosine kinase inhibitors
Qtc prolongation
Most common MPN
Essential Thrombocythemia
In Essential Thrombocythemia there is a marked increase in
Megakaryocytes and Platelets
Symptoms of Essential Thrombocythemia
erythromelalgia (red, painful hands and feet)
Livedo reticularis
Thrombosis
Mutation in ET
Jak2
Treatment for low risk ET
Aspirin
Treatment for high risk ET patients
Hydroxyurea + Aspirin
Emergent treatment for ET
Platelet pheresis
Hgb >16.5 in men; >16 in women
Polycythemia Vera
Mutation in PCV
Jak2
Microscopic hematuria + elevated hemoglobin and erythrocyte
erythropoitin hypernephroma
Treatment for PCV
Therapeutic phlebotomy. Goal Hct 45%
Hydroxyurea, Aspirin (low dose)
Hydroxyurea and Aspirin can treat
PCV
ET
CML (just hydroxyurea-palliative)
Budd chiari or portal vein thrombosis
think of PV
Primary myelofibrosis treatment
Supportive
HSCT for ppl <60
Hydroxyurea, ruxxolitinib (Jak2 inhibitor) for symptoms
