Myelodysplastic syndromes: 2011 update on diagnosis, risk-stratification, and management.

摘要: Disease overview: The myelodysplastic (MDS) are a very heterogeneous group of myeloid disorders characterized by peripheral blood cytopenias and increased risk transformation to acute myelogenous leukemia (AML). MDS occurs more frequently in older male individuals with prior exposure cytotoxic therapy. Diagnosis: Diagnosis is based on morphological evidence dysplasia upon visual examination bone marrow aspirate biopsy. Information obtained from additional studies such as karyotype, flow cytometry, or molecular genetics complementary but not diagnostic. Risk-stratification: Prognosis patients can be calculated using number scoring systems. In general, all these systems include analysis cytopenias, percentage blasts the marrow, cytogenetic characteristics. The most commonly used system International Prognostic Scoring System. This score divides into lower subset (low intermediate-1) higher (int-2 high). Other modern have been developed that allow precise calculation. Risk-adapted therapy: Therapy selected risk, transfusion needs, percent recently profile. Goals therapy different than risk. goal decrease needs disease AML. prolong survival. Current available therapies growth factor support, lenalidomide, hypomethylating agents, intensive chemotherapy, allogeneic stem cell transplantation. use lenalidomide has significant clinical activity disease, anemia, chromosome 5 alteration. 5-azacitidine decitabine MDS. shown improve survival Additional supportive care measures may prophylactic antibiotics iron chelation. Management progressive refractory disease: At present time, there no approved interventions for particularly after therapy. Options cytarabine-based therapy, transplantation, participation trial. Am. J. Hematol. 86:491–498, 2011. © 2011 Wiley-Liss, Inc.