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伴有20号染色体长臂缺失异常的原发性血小板增多症继发的铁粒幼细胞贫血

[Sideroblastic anemia preceded by essential thrombocythemia with 20q- chromosome abnormality].

作者信息

Yamada K, Nakamaki T, Yokoyama A, Hino K, Tomoyasu S, Sakurai M, Tsuruoka N

机构信息

Department of Hematology, Showa University School of Medicine.

出版信息

Rinsho Ketsueki. 1993 Sep;34(9):1027-32.

PMID:8230746
Abstract

A 78-year-old man presented with marked thrombocytosis (126.4 x 10(4)/microliters), low neutrophil alkaline phosphatase (NAP) score and an abnormal karyotype of 46, XY, del(20) (q11q13) (18 of 20 cells), without obvious anemia or ringed sideroblasts in bone marrow. He received ranimustine (MCNU) with a diagnosis of essential thrombocythemia. After 2 years, he was admitted because of macrocytic anemia. The peripheral blood smear showed anisopoikilocytosis with a few nucleated red blood cells. Moderate thrombocytosis (71.7 x 10(4)/microliters) and a low NAP score were also observed. Bone marrow aspiration revealed erythroid hyperplasia with a significant increase in ringed sideroblasts (85% of erythroblasts). Cytogenetic studies showed the same abnormal karyotype 46, XY, del(20) (q11q13) in 100% of metaphase cells as those at initial diagnosis. A diagnosis of sideroblastic anemia preceded by essential thrombocythemia was made. No rearrangement or amplification of c-src was revealed. The observation of the same chromosome abnormality (20q-) in different phases of this patient's disease indicates that chronic myeloproliferative disorders and myelodysplastic syndrome may share some borderline or transitional cases with a similar pathogenesis.

摘要

一名78岁男性患者,血小板显著增多(126.4×10⁴/微升),中性粒细胞碱性磷酸酶(NAP)评分低,核型异常为46, XY, del(20)(q11q13)(20个细胞中有18个),骨髓中无明显贫血或环形铁粒幼细胞。他被诊断为原发性血小板增多症并接受了雷莫司汀(MCNU)治疗。2年后,他因大细胞性贫血入院。外周血涂片显示红细胞大小不均和异形红细胞增多,可见少量有核红细胞。还观察到中度血小板增多(71.7×10⁴/微升)和低NAP评分。骨髓穿刺显示红系增生,环形铁粒幼细胞显著增加(占幼红细胞的85%)。细胞遗传学研究显示,100%的中期细胞核型与初诊时相同,为46, XY, del(20)(q11q13)。诊断为原发性血小板增多症继发的铁粒幼细胞贫血。未发现c-src重排或扩增。该患者疾病不同阶段均观察到相同的染色体异常(20q-),表明慢性骨髓增殖性疾病和骨髓增生异常综合征可能存在一些发病机制相似的临界或过渡病例。

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