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🌱 來自: myelodysplastic syndromes

myelodysplastic syndromes (MDS) overview

(Lancet 2014;383:2239)

• Acquired clonal stem cell disorder → ineffective hematopoiesis → cytopenias, dysmorphic blood cells and precursors, variable risk of leukemic transformation

• Epidemiology: 20–30,000 cases/y; median age ~70 y; male predominance (1.8×)

• Idiopathic or 2° to chemo w/ alkylating agents; ↑ risk w/ radiation, benzene

• Clinical manifestations:

  • anemia (85%),
  • neutropenia (50%),
  • thrombocytopenia (40–65%)

• Diagnosis:

  • dysplasia (usually multilineage) in peripheral smear
    • oval macrocytes,
    • pseudo-Pelger-Huët anomaly
  • and bone marrow (≥10% dysplasia with blasts ± RS)

• Both

  • cytogenetic [eg, del(5q), mono 7, del(7q), trisomy 8, del(20q)] and
  • molecular abnl (TP53, EZH2, ETV6, RUNX1, ASXL1, SF3B1, DNMT3A) may carry prognostic signif

• Prior to dx MDS:

  • exclude AML (≥20% blasts) and
  • CMML (monos >1 × 109/L);
  • r/o 2° BM Δs (defic. of B12, folate, copper);
  • viral infxn (eg, HIV); EtOH; lead, arsenic exposures