骨髓增生异常综合征 .PPT

临床表现 差异大、早期低危患者无症状 贫血 发热、感染 出血 一般无肝脾淋巴结肿大 转化为急性白血病 老年患者多有合并症 Recommendations and Definitions in MDS Recommendations Differential: 500 in BM, 200 cells in PB Number: 200 for G and E, 30 cells for meg. Ring sideroblasts: ≥ 5 iron granules encircling ≥ 1/3 of the nucleus Minimal dysplastic changes (good quality of smear) 10% in one single cell line* or 10% with recurrent abnormal cytogenetics Cytopenia (≥ 6 month), Transfusion-dependent, macrocytic anemia Hgb 10g/dL ANC 1.5 x 109/L PLT 100 x 109/L Constant blast count 5-19% * Morphologic Features Blasts：Myeloblasts Dysmyelopoiesis; Dyserythropoiesis; Dysgranulopoiesis; Dysmegakaryopoiesis Trephine biopsy: Cellularity; Myelofibrosis (reticulin, MDS with fibrosis); Report estimated % of CD34+ blasts; Dysmegakaryopoiesis (CD61); ALIP(abnormal localized immature precursors) * 红系病态造血 Normal Ringed sideroblasts * 粒系病态造血 * 巨核系病态造血 * 诊断、鉴别诊断 PB：cytopenia(s) BM smear：dysplasia BM biopsy：ALIP Flow cytometry Cytogenetic：5q-/-5, -7 Molecular：NGS MA, AA, PNH, MPN, AML * AML with less than 20% blasts MDS with PNH features AML FAB M6 when erythroblasts are 50% MDS with hypocellular marrow MDS with fibrosis MDS with thrombocytosis PNH MPN AA MDS AML * Minimal Diagnostic Criteria in MDS Prerequisite criteria Constant cytopenia in one or more of the following cell lineages: erythroid (hemoglobulin 11 g dL-1); or neutrophilic (ANC1500 μ-1); or megakaryocytic (platelets 100,000 μL-1) Exclusion of all other hematopoietic or non-hematopoietic disorders as primary reason for cytopenia/dysplasiab MDS-related (decisive) criteria Dysplasia in at least 10% of all cells in one of the following lineages in the bone marrow smear; erythroid; neutrophilic; or megakaryocytic or 15% ringed sideroblasts 5-19% Blast cells in bone marrow smears Typical chromosomal abnormality: conventional karyotyping or FISH Valent P, et al. Leukemia Research 2007:727-736 * Minimal Diagnostic Criteria in MDS Cont’d... (C)