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一例伴有18号长臂等臂染色体的骨髓增生异常综合征的临床和实验观察
引用本文:陈钟灵,薛永权,杨复,曹幼甫,过宇,谢新,陆定伟. 一例伴有18号长臂等臂染色体的骨髓增生异常综合征的临床和实验观察[J]. 苏州大学学报(自然科学版), 1994, 0(6)
作者姓名:陈钟灵  薛永权  杨复  曹幼甫  过宇  谢新  陆定伟
作者单位:江苏省血液研究所
摘    要:报告1例伴有i(18q)和骨髓纤维化的骨髓增生异常综合征。患者为青年男性,有贫血史十余年,近来脾进行性肿大,骨髓多次干抽。血象示全血细胞减少,有核红细胞显著增多和原粒细胞轻度增加。骨髓涂片和活检示有核细胞增生活跃和涉及三系的病态造血,网状纤维明显增生而无胶原纤维沉着。骨髓细胞染色体R带核型分析发现,83%的中期细胞有i(18q)。患者死于骨髓衰竭所致的严重贫血和出血。我们认为,i(18q)和骨髓纤维化可能与本例的不良预后有关。

关 键 词:骨髓增生异常综合征  i(18q)  诊断

AnISochromosomeoftheLongArmofChromsome18inpatientwithMyelodysplasticSyndromewithMyelofibrosis
ChenZhongling,etal. AnISochromosomeoftheLongArmofChromsome18inpatientwithMyelodysplasticSyndromewithMyelofibrosis[J]. Suzhou University Journal of Medical Science, 1994, 0(6)
Authors:ChenZhongling  etal
Abstract:hisarticlereportsthatapatientofmyelodysplasticsyndrome(MDS)withmyelofibrosisandi-(18q).ThePatientwhoisa29--yeal--oldman,waSnotedtobeanemicover10--years.Recentlythespleenbecameprigressuvektmassiveandbonemarowaspiratesyielded"dytaps"onseveraloccasions.Hematologicalinvestigationdisclosedpancytopenia,numerousnucleatedredcellsandmildiyincreasedmyloblasts(8%)intheperipheralblood.Bonemarrowaspirateandbiopsyrevealedhypercellularmarrow,trilineagemyelodysplasia,significantreticulinfibrosis,,butwithoutcollagenformation.BonemarrowtwotypicanalysiswithR-bandingshowedandisochromosome18qassoleabnormalityintwentyoutof24metaphasesanalysed.Atlast,hediedofsevereanemiaandbleedingduetoboonemarrowfailure.Weconsiderthati(18q)acmyelofibrosismayberelatedtohisPoorprognosis.
Keywords:Myelodysplasicsyndrome  refractoryanemia  Myelofibrosis  cytogenetics  isochromosome
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