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一例骨髓增生异常综合征(MDS)的尸检病例:MDS与其他造血系统疾病(包括急性骨髓纤维化)之间的诊断问题

An autopsy case of myelodysplastic syndrome (MDS): diagnostic problems between MDS and the other haematopoietic disorders including acute myelofibrosis.

作者信息

Hayashi Y, Tange T, Chi L C, Ishida T, Kitamura T, Kitamura K, Urano Y

出版信息

Nihon Ketsueki Gakkai Zasshi. 1989 Feb;52(1):49-54.

PMID:2741651
Abstract

We report an autopsy case of myelodysplastic syndrome (MDS) in a 35-year-old male, who presented with pancytopenia and bleedings. Bone marrow specimens disclosed myelofibrosis and hypercellular marrow with more than 60% atypical erythroblasts in the bone marrow cells. Type I or type II blasts were less than 10% of the peripheral blood and bone marrow cells during the clinical course. At autopsy, infiltration by myeloid and erythroid cells and megakaryocytes was noted in the liver, spleen and lymph nodes. According to the FAB classification, this case might be classified into refractory anemia with excess of blasts (RAEB) or RAEB in transformation. However, the remarkable neoplastic proliferation of three haematopoietic cell lines also indicates acute myeloproliferative disorder such as acute myelofibrosis or acute panmyelosis.

摘要

我们报告一例35岁男性骨髓增生异常综合征(MDS)的尸检病例,该患者表现为全血细胞减少和出血。骨髓标本显示骨髓纤维化,骨髓细胞增多,骨髓细胞中非典型成红细胞超过60%。在临床过程中,I型或II型原始细胞在外周血和骨髓细胞中所占比例小于10%。尸检时,在肝脏、脾脏和淋巴结中发现有髓系、红系细胞和巨核细胞浸润。根据FAB分类,该病例可能归类为伴有过多原始细胞的难治性贫血(RAEB)或转化中的RAEB。然而,三种造血细胞系显著的肿瘤性增殖也提示急性骨髓增殖性疾病,如急性骨髓纤维化或急性全髓增殖症。

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