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发育不全性骨髓疾病患者磁共振成像显示克隆性疾病的证据。

Evidence for clonal disease by magnetic resonance imaging in patients with hypoplastic marrow disorders.

作者信息

Negendank W, Weissman D, Bey T M, de Planque M M, Karanes C, Smith M R, Ratanatharathorn V, Bishop C R, al-Katib A M, Sensenbrenner L L

机构信息

Department of Medicine, Harper Hospital, Wayne State University, Detroit, MI.

出版信息

Blood. 1991 Dec 1;78(11):2872-9.

PMID:1954375
Abstract

Some patients with hypoplastic marrow disorders, including aplastic anemia (AA), are at risk for clonal evolution to myelodysplastic syndromes (MDS) and leukemia. Magnetic resonance imaging (MRI) of marrow of the spine, pelvis, and femurs was performed in 24 patients with hypoplastic marrow disorders. In 12 patients (three AA, nine MDS) MRI was compatible with the clinical and biopsy diagnoses and served to define the spectrum of marrow patterns in these disorders. In eight patients with hypocellular marrow biopsies and a clinical diagnosis of AA, MRI showed an unexpected inhomogeneous or diffuse cellular pattern. Concurrent or subsequent marrow or cytogenetic studies have led to diagnoses of hypoplastic MDS in seven of these patients. In four patients with prolonged hypoplasia after bone marrow transplantation for lymphoma, a speckled pattern superimposed on a fatty background appeared in serial MRI studies. One case evolved to AML, two developed megaloblastic foci, and one remains hypoplastic at 19 months. This study suggests that MRI is able to detect early clonal disease in patients with AA, and can distinguish AA from hypoplastic MDS.

摘要

一些患有骨髓发育不全疾病(包括再生障碍性贫血(AA))的患者有向骨髓增生异常综合征(MDS)和白血病克隆演变的风险。对24例骨髓发育不全疾病患者进行了脊柱、骨盆和股骨骨髓的磁共振成像(MRI)检查。在12例患者(3例AA,9例MDS)中,MRI与临床和活检诊断相符,并有助于确定这些疾病的骨髓模式范围。在8例骨髓活检细胞减少且临床诊断为AA的患者中,MRI显示出意外的不均匀或弥漫性细胞模式。同时或随后的骨髓或细胞遗传学研究导致其中7例患者被诊断为发育不全性MDS。在4例因淋巴瘤接受骨髓移植后长期发育不全的患者中,系列MRI研究显示在脂肪背景上叠加有斑点状模式。1例演变为急性髓系白血病(AML),2例出现巨幼细胞灶,1例在19个月时仍处于发育不全状态。这项研究表明,MRI能够检测出AA患者的早期克隆性疾病,并能将AA与发育不全性MDS区分开来。

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