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14 Cards in this Set

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Cardinal features of MDS
LOW peripheral blood cell counts
DIMINISHED cell production (ineffective hematopoiesis)
COMPLETE maturation, BUT often abnormal (dysplasia)
LESS THAN 20% blasts
MDS comparative characteristics
Mostly MATURE cells
HIGH proliferation rate (BUT, increased apoptosis)
DYSPLASIA in 75% of cases
Onset is GRADUAL
Untreated MAY SURVIVE FOR YEARS
RELATIVELY RESISTANT to chemo
Characteristics of LOW GRADE MDS
Anemia only
Mild to no dysplasia
Chromosomes normal or 5q-
NO Auer Rods
RARELY progresses to AML
Prognosis is GOOD
Treatment is SUPPORTIVE
Characteristics of HIGH GRADE MDS
MULTI-LINEAGE cytopenia
MULTI-LINEAGE dysplasia
+/- Auer rods
Chromosomes complex, -7/-7q
COMMONLY progresses to AML
Prognosis is POOR
Treatment entails CHEMO
Median age for MDA
~70 y.o.
Predisposing factors for MDS
Age
DNA damaging agents
Hereditary -- FANCONI's ANEMIA

(These are in common w/ AML)
5q- Syndrome
Loss of LONG arm of chromosome 5

MACROcytic anemia
NORMAL-increased platelets, HYPOlobulated megakaryocytes

Pts. have a very long survival (LOW grade)
Frequency of translocations in MDS
VERY RARE

Translocations usually imply acute leukemia
~20% of MDS has mutations in this
Ras family (signaling)
Relative survival rates of MDS
Low grade -- median of at least 6 yrs.

High grade:
Refractory anemia w/ excess blasts -- ~1 yr.
Refractory anemia w/ multi-lineage dysplasia -- ~2.5 yrs.
Causes of death in MDS
BM Failure -- 60%
Progression to AML -- 40%
Negative prognostic factors for MDS
Increased numbers of blasts
Unfavorable karyotype -- complex, -7/-7q
Multi-lineage cytopenia
Treatment for LOW GRADE MDS
Supportive
Occasional RBC transfusions
Treatment for HIGH GRADE MDS
Chemo has LIMITED efficacy
Stem cell transplant only for younger and healthier

Treatment of neutropenia w/ G-CSF