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
What is the most common complication of PV?
Treatment?
Thrombosis
Phlebotomy
What MDS type has the lowest risk of conversion to AML (10-15%)?
Refractory Anemia w/Ringed Sideroblasts
Leukoerythroblastic anemia, poikilocytosis and splenomegaly are characteristic of what?
What classic clinical feature?
Primary myelofibrosis
Dry tap on bone marrow aspirate
What is the Etiology of MDS?
Chemo Tx for breast cancer, HL, NHL, Acute leukemia Anthracyclines, alkylating agents Radiation Oil and shipyard workers
What new drug is approved for tx of intermediate and high risk myelofibrosis?
Ruxolitinib
What is the disease course for PM?
Chronic but progressive
Pts w/PM and splenomegaly, what are the 3 treatments in progressive intensity?
Hydroxyurea
Radiation
Splenectomy
Describe the general MDS clinical picture
Pancytopenia w/hyperplastic marrow
What cytogenetics give a favorable prognosis in MDS?
What drug has a good effect in this type?
5q-
Lenalidomide (angiogenesis inhibitor)
