Vila L, Charrin C, Archimbaud E, Treille-Ritouet D, Fraisse J, Felman P, Fiere D, Germain D
Laboratoire Central d'Hématologie et de Cytogénétique, Hôpital Edouard Herriot, Lyon, France.
Blut. 1990 Apr;60(4):223-7. doi: 10.1007/BF01728788.
In order to detect possible relationships between cytogenetic abnormalities and morphologic features in myelodysplastic syndromes (MDS), 48 patients with MDS were investigated. Clonal cytogenetic abnormalities were present in bone marrow cells from 27 patients (56%). The most frequent single anomaly was del (5 q) (10 cases), followed by monosomy 7 (3 cases), trisomy 8 (3 cases) and del (20 q) (2 cases). Complex anomalies were present in 6 patients. Morphologically, according to the French-American-British (FAB) classification: 17 cases were considered as refractory anemia (RA), 17 as RA with excess of blasts (RAEB), 2 as RAEB in transformation, 2 as acquired idiopathic sideroblastic anemia and 10 as chronic myelomonocytic leukemia. With regard to the FAB classification, del (5 q) was often associated with RA and complex cytogenetic anomalies with RAEB. When myelodysplasia was studied in individual myeloid lineages, del (5 q) was associated with hypolobulated megakaryocytes, monosomy 7 with micromegakaryocytes and complex chromosomal anomalies with the association of two or more features of dysmegakaryocytopoiesis. Del (11 q) was associated with increased iron storage and del (20 q) with marked dyserythropoiesis. No correlation was observed between cytogenetic anomalies and features of dysgranulocytopoiesis.
为了检测骨髓增生异常综合征(MDS)中细胞遗传学异常与形态学特征之间可能存在的关系,对48例MDS患者进行了研究。27例患者(56%)的骨髓细胞存在克隆性细胞遗传学异常。最常见的单一异常是del(5q)(10例),其次是7号染色体单体(3例)、8号染色体三体(3例)和del(20q)(2例)。6例患者存在复杂异常。形态学上,根据法美英(FAB)分类:17例被认为是难治性贫血(RA),17例是伴有原始细胞增多的难治性贫血(RAEB),2例是转化中的RAEB,2例是获得性特发性铁粒幼细胞贫血,10例是慢性粒单核细胞白血病。关于FAB分类,del(5q)常与RA相关,复杂细胞遗传学异常与RAEB相关。当对单个髓系进行骨髓增生异常研究时,del(5q)与核分叶过少的巨核细胞相关,7号染色体单体与小巨核细胞相关,复杂染色体异常与双核或更多核异常特征的巨核细胞生成相关。del(11q)与铁储存增加相关,del(20q)与明显的红细胞生成异常相关。未观察到细胞遗传学异常与粒细胞生成异常特征之间的相关性。