骨髓增生异常综合征诊治-英文2011.pptVIP

Myelodysplastic syndromes:A practical approach to diagnosis and treatment Hong-ling Hao, M.D. Department of Hematology, Hebei General Hospital Introduction MDS are a heterogeneous group of disorders of blood cell production in the bone marrow that can transform into acute myeloid leukemia (AML). They are diagnosed most often in the elderly. The consequent cytopenias result in infections and bleeding complications. The range of symptoms depends on the bone marrow cell type affected. They have symptoms stemming from anemia or thrombocytopenia, or have recurrent infections. Subtypes of MDS have different pathologic and clinical presentations and different prognoses. They are often categorized as lower-risk or higher-risk, depending on the likelihood of transforming to AML. Lower-risk MDS survive a median of 3 to 7 years. Higher-risk types are pathobiologically similar to AML in older adults, and patients either develop AML or die of complications of MDS, on average within 1.5 years. Etiology Genetic and environmental factors probably both play a role. Environmental factors: smoking; ionizing radiation; exposure to benzene, hair dyes, pesticides. Genetic factors: Down syndrome, Fanconi anemia, and Bloom syndrome are associated with MDS. MDS rarely run in families. Diagnosis——Laboratory tests Complete blood cell count (CBC) Evaluation of anemia Tests for nutrient deficiencies Fecal occult blood testing Liver function tests Renal function tests Thyroid function tests Reticulocyte count EPO level ? Screening for relevant infections: HIV, Hepatitis, Parvovirus B19…… ? Review of drugs: MTX, Depakote, Luminal, Retrovir…. Evaluation of other cytopenias Abdominal ultrasonography ---- for splenomegaly Coombs antiglobulin tests LDH level Antinuclear antibody titer Rheumatoid factor level If tests are negative Peripheral blood smear Bone marrow