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真性红细胞增多症耗竭期并发多发性骨髓瘤和急性巨核细胞白血病

Multiple myeloma and acute megakaryoblast leukemia in spent phase polycythemia vera.

作者信息

Terpstra W E, Meuwissen O J, Hagemeijer A, Michiels J J

机构信息

Department of Internal Medicine, St. Antonius Hospital, Nieuwegein, The Netherlands.

出版信息

Am J Clin Pathol. 1990 Dec;94(6):786-90. doi: 10.1093/ajcp/94.6.786.

Abstract

The spontaneous and simultaneous occurrence of multiple myeloma and megakaryoblast leukemia with myelodysplastic features in a case of spent phase polycythemia vera is well documented. In support of the morphologic characteristics of the bone marrow, immunocytologic studies show proliferation of monoclonal plasma cells and megakaryoblasts. The cytogenetic findings of 20q- and unbalanced t(1;7) are consistent with myelodysplastic and leukemic transformation of the bone marrow. These transformations expand observations on variable and spontaneous lineage commitments as the consequence of alterations of the hematopoietic stem cell clone. These data are in support of the changing insights in hematopoiesis as a process of ordered commitment of the stem cell with sequential lineage potentials.

摘要

在一例终末期真性红细胞增多症患者中,同时自发出现多发性骨髓瘤和具有骨髓发育异常特征的巨核母细胞白血病,这一情况已有充分记录。为支持骨髓的形态学特征,免疫细胞研究显示单克隆浆细胞和巨核母细胞增殖。20q-和不平衡t(1;7)的细胞遗传学发现与骨髓的骨髓发育异常和白血病转化一致。这些转化扩展了对造血干细胞克隆改变导致可变且自发谱系定向的观察。这些数据支持了对造血过程的不断变化的认识,即造血是一个干细胞按顺序具有谱系潜能的有序定向过程。

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