MDS, MPSs - Hubbard Flashcards
MDS are disorders of what?
Characterized by what?
Pluripotent stem cell
Ineffective hematopoiesis
Pts w/HA, dizziness, tinnitus, blurred vision, and fatigue most likely have what?
What other common symptom?
PV
Hyperviscosity
What subset of pts may benefit from androgen therapy for MDS?
Hypoplastic marrow pts
What cytogenetics give a poor prognosis in MDS?
Monosomy 7
HypOdiploidy
multiple abn.
treatment of MDS?
Supportive care and aggressive tx of infections
What is the only curative tx for PM?
Allogenic stem cell transplant
What is the tx for MDS in high intensity therapy?
AML style, not as effective as de novo AML
HSC transplant if under 60 and has HLA matched sibling donor
What is the pathogenesis of myelofibrosis?
Increased reticulin deposition in the marrow
2ndary to increased PDGF
What labs are abnormal in MDS?
Inc LDH
Iron overload
Inc ferritin
What are the adverse prognosis features of MDS?
Marrow blasts > 5% Platelets < 100,000 Hb < 10 Neutrophils < 2500 Age > 60
What mutation is present 45-65% of PM?
JAK2
What is the most common cause of death in PM?
Overwhelming infection
When is EPO ineffective in pts with MDS?
EPO > 500
Describe the following in PV:
Hct?
EPO?
High
Low
What is the tx for low/intermediate intensity therapy for MDS?
Hypomethylating agents like azacitidine and decitabine