MDS Flashcards

1
Q

epidemiology

A

median onset 7th decade,

60% men

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2
Q

cytogenetics standard (testable)

A

5q- only 25% progress to leukemia (low frequency, better survival);

other cytogenetics probably not testable

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3
Q

5q- syndrome -6

A

refractory anemia,

mild leukop,

atypical megakaryocytes,

normal to elevated plts,

transfusion dependence,

extended survival

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4
Q

clinical sx

A

fever, pallor, infx, bruising, fatigue, dyspnea on exertion, bleeding

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5
Q

lab sx. -5

A

macrocytic anemia,

neutrop,

thrombocytop,

nucleated RBC,

peripheral blasts

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6
Q

classification systems

A

WHO replaced FAB

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7
Q

international prognostic scoring system for MDS variables -3

A

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

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8
Q

factors to consider for treatment

A
IPSS score, 
WHO/FAB classification
age
PS
HLA-DR status
cytogenetic status
comorbidity
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9
Q

goal of hypomethylating agents

A

delay transformation to AML, NOT improve OS

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10
Q

tx options for MDS (5pts)

A
  1. erythropoietic agents (epo, darbe, g-csf, gm-csf)
  2. supportive measures (transfusion, chelation tx)
  3. hypomethylating agents (azacitidine, decitabine)
  4. immunomodulation drugs (lenalid FDA approved for 5q-)
  5. SCT / induction chemo
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11
Q

management of anemia - transfusions (5pts)

A

most pt’s require,

goal hgb >10,

iron chelation (defuroxime) needed if >20-30 units,

infx complications,

dec OS if transfusion dependent

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12
Q

iron chelation therapy indx -6 and Duration -1

A
  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

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13
Q

ECOG phase III EPO trial

A

epo 150units/m2/d -> if NO response EPO + gcsf 1mg/kg/d ->if NO response EPO 300units/m2/d + gcsf

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14
Q

azacitidine vs supportive care (BSC) and vs induction chemo pre HCT and vs HCT

A

75mg/m2 SC or IV D1-7 q28d

#BSC: CR 7% vs 0, 
OS 24.5 vs 16mo (p=sig)
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15
Q

decitibine vs BSC

A

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)
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16
Q

lenalidomide (in 5q- pts ONLY) - phase II trial

A

better median Hgb, CCyR, PCyR

17
Q

alloSCT

A

high risk up front, better long term OS