MDS Flashcards
epidemiology
median onset 7th decade,
60% men
cytogenetics standard (testable)
5q- only 25% progress to leukemia (low frequency, better survival);
other cytogenetics probably not testable
5q- syndrome -6
refractory anemia,
mild leukop,
atypical megakaryocytes,
normal to elevated plts,
transfusion dependence,
extended survival
clinical sx
fever, pallor, infx, bruising, fatigue, dyspnea on exertion, bleeding
lab sx. -5
macrocytic anemia,
neutrop,
thrombocytop,
nucleated RBC,
peripheral blasts
classification systems
WHO replaced FAB
international prognostic scoring system for MDS variables -3
cytogentics, %BM blasts, Hgb, Plt, ANC
ranges from 0.8 yrs to 8.8yrs OS v.high risk to v.low,
time to 25% AML conversion increases with score from 0.73yrs to 10.8yr for low and NOT reached for v. low
factors to consider for treatment
IPSS score, WHO/FAB classification age PS HLA-DR status cytogenetic status comorbidity
goal of hypomethylating agents
delay transformation to AML, NOT improve OS
tx options for MDS (5pts)
- erythropoietic agents (epo, darbe, g-csf, gm-csf)
- supportive measures (transfusion, chelation tx)
- hypomethylating agents (azacitidine, decitabine)
- immunomodulation drugs (lenalid FDA approved for 5q-)
- SCT / induction chemo
management of anemia - transfusions (5pts)
most pt’s require,
goal hgb >10,
iron chelation (defuroxime) needed if >20-30 units,
infx complications,
dec OS if transfusion dependent
iron chelation therapy indx -6 and Duration -1
- INDICATIONS:
serum ferritin reaches 1000 mg/L,
transfusion 2x/mo for at least 1 yr (ie. transfusion dependent),
low risk MDS (as will live longer),
need to preserve organ function,
alloSCT candidates (will need more transfusions),
life expectancy greater than 1yr
—con’t as long as needed, agent based on availability, reimbursement
ECOG phase III EPO trial
epo 150units/m2/d -> if NO response EPO + gcsf 1mg/kg/d ->if NO response EPO 300units/m2/d + gcsf
azacitidine vs supportive care (BSC) and vs induction chemo pre HCT and vs HCT
75mg/m2 SC or IV D1-7 q28d
#BSC: CR 7% vs 0, OS 24.5 vs 16mo (p=sig)
decitibine vs BSC
ROLE: if can’t tolerate more intense chemo= older pts, more comorbidities
#CR 9% vs 0, median duration response 9.5 vs 7.6mo, OS (NS), AML at 1 yr 22 vs 33% (sig), PFS 6.6 vs 3mo (sig)