MDS的诊断与治疗.ppt

MDS的诊断与治疗.ppt中国医学科学院血液学研究所血液病医院
秦铁军电话:**********
骨髓增生异常综合征
Thursday, September 06, 2018
MDS的定义与最低诊断标准
定义
a group of clonal haematopoietic stem cell diseases
characterized by
cytopenia(s).
dysplasia in one or more of the major myeloid cell lines.
Ineffective haematopoiesis
Increased risk of development of AML
Monday, August 09, 2010
thresholds for cytopenias
Cytopenias
Haemoglobin < 100g/L
Absolute neutrophil count (ANC)<
Platelets < 100xl09/L
Definitive morphologic and/or ic findings support the diagnosis of MDS even if values above these thresholds
Monday, August 09, 2010
流行病学(Epidemiology)
Median age 70 years
incidence of 3-5/100 000 persons
>20/100 000 among those over the age of 70 years
male predominance
Clinical features
Symptoms related to cytopenia(s)
Anemic and transfusion-dependent
Neutropenia and/or thrombocytopenia --Less frequent
anomegaly
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The morphological of MDS
principally based on
percent of blasts in the BM and PB
type and degree of the dysplasia
presence of ring sideroblasts
500-cell differential of all nucleated cells in the BM smear
200-leukocyte differential in the PB
necessity of high quality slide preparations and the stain
Monday, August 09, 2010
骨髓和外周血涂片
高质量的制片和染色
新鲜标本(抗凝标本不应超过2小时)
计数500个细胞
粒红比≤1:1时
计数500个非红系细胞,不包括淋巴细胞、浆细胞、肥大细胞
原始细胞比例应为非红系比例
环形铁粒幼细胞比例>15%时,即认为是红系发育异常
Monday, August 09, 2010
Morphologic manifestation of dysplasia(1)
Dyserythropoiesis
Nuclear
核出芽(nuclear budding)
核间桥(internuclear bridging)
核破裂(Karyorrhexis)
多核(Multinuclearity)
核多分叶(nuclear hyperlobation)
巨幼样变(megaloblastic changes)
Cytoplasmic
环状铁粒幼红细胞(Ring sideroblast)
空泡形成(Vacuolization)
糖原染色阳性(Periodic acid-Schiff positivity)
Monday, August 09, 2010
Morphologic manifestation of dysplasia(2)
Dysgranulopoiesis
细胞体积过小或过大(Small or unusually large size)
核少分叶Nuclear hypolobation(pseudo Pelger-Huet; pelgeroid)
不规则的分叶过多(Irregular hypersegmentation)
少颗粒(Decreased granules; agranularity)
Pseudo Chediak-Higashi granules
奥氏小体Auer ro