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血管内淋巴瘤伴骨髓受累及白血病期表现。

Intravascular lymphoma presenting with bone marrow involvement and leukemic phase.

作者信息

Khoury Haytham, Dalal Bakul I, Nantel Stephen H

机构信息

Leukemia/BMT Program of British Columbia, Vancouver General Hospital and Health Sciences Center, Vancouver, Canada.

出版信息

Leuk Lymphoma. 2003 Jun;44(6):1043-7. doi: 10.1080/1042819031000067530.

Abstract

We describe the case of a 62-year-old man with recent onset of constitutional symptoms and vague intellectual deficit. The blood showed pancytopenia with blastemia, and bone marrow confirmed an extensive "vacuolated blast-like cell" infiltrate. Initial diagnosis of, and treatment for Burkitt's leukemia/lymphoma was questioned when the "blasts" typed as CD5+ mature B-cells; however, it was revised to intravascular lymphoma (IVL) only after the sinusoidal pattern was confirmed by immunocytochemistry. Literature review indicated that blood and bone marrow involvement in IVL appears to be rare, but a systematic search for this involvement is often not carried out. CD5 expression has been increasingly reported in this disease. The actual frequency and the significance of this expression are still to be defined.

摘要

我们描述了一名62岁男性的病例,他近期出现全身症状和模糊的智力缺陷。血液检查显示全血细胞减少伴原始细胞血症,骨髓检查证实有广泛的“空泡状原始细胞样细胞”浸润。当“原始细胞”被鉴定为CD5+成熟B细胞时,最初对伯基特白血病/淋巴瘤的诊断和治疗受到质疑;然而,只有在免疫细胞化学证实为窦状模式后,才将诊断修订为血管内淋巴瘤(IVL)。文献综述表明,IVL累及血液和骨髓似乎很少见,但通常不会对这种累及进行系统的检查。越来越多的报道称这种疾病中存在CD5表达。这种表达的实际频率及其意义仍有待确定。

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