Myelodysplastic syndromes

項目內容
時間2014-05-22的14:00至15:00
類型丙類
積分1
主辦單位高雄榮民總醫院內科部
會議地點第二會議室
聯絡人:李沅融、鍾柏正、翁雅為
電話:(07) 3422121 ext 2085
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The myelodysplastic syndromes (MDS) comprise a heterogeneous group of malignant hematopoietic stem cell disorders characterized by dysplastic and ineffective blood cell production. MDS occurs most commonly in older adults and may occur de novo or arise years after exposure to potentially mutagenic therapy (eg, radiation exposure, chemotherapy). The diagnosis of MDS should be considered in any patient with unexplained cytopenia(s) or monocytosis. Careful inspection of the peripheral blood smear and bone marrow aspirate is necessary to document the requisite dysplastic cytologic features identifiable in any or all of the hematopoietic lineages (table 2). Detection of certain chromosomal abnormalities distinguishes between MDS and acute myeloid leukemia (AML) in some cases, aids in the classification of MDS, and is a major factor in determining prognostic risk group and therapy. The diagnosis of MDS requires both of the following: Otherwise unexplained quantitative changes in one or more of the blood and bone marrow elements (ie, red cells, granulocytes, platelets). The values used to define cytopenia are: hemoglobin <10 g/dL (100 g/L); absolute neutrophil count <1.8 x 109/L (<1800/microL); and platelets <100 x 109/L (<100,000/microL). However, failure to meet the threshold for cytopenia does not exclude the diagnosis of MDS if there is definite morphologic evidence of dysplasia. Morphologic evidence of significant dysplasia (ie, ≥10 percent of erythroid precursors, granulocytes, or megakaryocytes) upon visual inspection of the peripheral blood smear, bone marrow aspirate, and bone marrow biopsy in the absence of other causes of dysplasia (table 2). In the absence of morphologic evidence of dysplasia, a presumptive diagnosis of MDS can be made in patients with otherwise unexplained refractory cytopenia in the presence of certain genetic abnormalities. Importantly, blast forms must account for less than 20 percent of the total cells of the bone marrow aspirate and peripheral blood. In addition, the presence of myeloid sarcoma or certain genetic abnormalities, such as those with t(8;21), inv(16), or t(15;17), are considered diagnostic of acute myeloid leukemia, irrespective of the blast cell count. MDS must be distinguished from other entities that may also present with cytopenias and/or dysplasia. Common conditions that present with features similar to MDS include HIV infection, deficiencies of vitamin B12, folate, or copper, and zinc excess. Other entities considered in a specific case depend largely upon the presenting features. MDS is classified using the WHO classification system based upon a combination of morphology, immunophenotype, genetics, and clinical feature.