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外周T细胞淋巴瘤伴扩大的外套层区受累。

Peripheral T-cell lymphoma with involvement of the expanded mantle zone.

作者信息

Ikonomou Ida Münster, Tierens Anne, Troen Gunhild, Aamot Hege Vangstein, Heim Sverre, Lauritzsen Grete F, Vålerhaugen Helen, Delabie Jan

机构信息

Department of Pathology, The Norwegian Radium Hospital, University of Oslo, Oslo, Norway.

出版信息

Virchows Arch. 2006 Jul;449(1):78-87. doi: 10.1007/s00428-005-0123-z. Epub 2006 Apr 22.

DOI:10.1007/s00428-005-0123-z
PMID:16633785
Abstract

Peripheral T-cell lymphoma (PTCL) with a nodular architecture is rare. Recently, two variants have been described with infiltration of the B-cell follicle, one variant that localizes to the marginal zone with a so-called perifollicular growth pattern, and a variant that localizes to the germinal center. These lymphomas have a CD4+ phenotype and may express Bcl-6. We have studied five similar cases of PTCL with involvement of the B-cell follicle. However, our cases differ from the cases previously described by their predominant and frequently patchy involvement of the expanded mantle zone of the B-cell follicle at onset. Later biopsies in three of the cases show diffuse infiltration of the lymph node, without features of angioimmunoblastic TCL (AILT). All cases expressed Bcl-6 in addition to CD4. Cytogenetics was available in four of the cases but revealed no recurrent chromosomal aberrations or changes associated with other types of PTCL. No mutations of the BCL-6 gene were observed. Together, the cases seem to have an intermediately aggressive clinical behavior. Whether our cases are part of a spectrum of PTCLs that encompasses previously described variants with predominant marginal zone or germinal center infiltration or they represent a separate T-cell lymphoma type remains to be demonstrated by a study of more of such cases.

摘要

具有结节状结构的外周T细胞淋巴瘤(PTCL)较为罕见。最近,已经描述了两种伴有B细胞滤泡浸润的变异型,一种定位于边缘区,具有所谓的滤泡周围生长模式,另一种定位于生发中心。这些淋巴瘤具有CD4+表型,可能表达Bcl-6。我们研究了5例类似的伴有B细胞滤泡受累的PTCL病例。然而,我们的病例与先前描述的病例不同,其在发病时主要累及并常呈斑片状累及B细胞滤泡的扩大套区。其中3例病例后来的活检显示淋巴结弥漫性浸润,无血管免疫母细胞性TCL(AILT)特征。所有病例除表达CD4外还表达Bcl-6。4例病例可进行细胞遗传学分析,但未发现复发性染色体畸变或与其他类型PTCL相关的改变。未观察到BCL-6基因的突变。总体而言,这些病例似乎具有中度侵袭性的临床行为。我们的病例是包含先前描述的以边缘区或生发中心浸润为主的PTCL变异型的PTCL谱系的一部分,还是代表一种单独的T细胞淋巴瘤类型,仍有待通过对更多此类病例的研究来证实。

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Blood. 2002 Nov 1;100(9):3369-73. doi: 10.1182/blood.V100.9.3369.
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Hemophagocytic syndrome: a rare complication of allogeneic nonmyeloablative hematopoietic stem cell transplantation.
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Peripheral T-cell lymphomas of follicular helper T-cell type frequently display an aberrant CD3(-/dim)CD4(+) population by flow cytometry: an important clue to the diagnosis of a Hodgkin lymphoma mimic.滤泡辅助性T细胞型外周T细胞淋巴瘤通过流式细胞术常显示异常的CD3(- / 弱阳性)CD4(+)细胞群:这是诊断霍奇金淋巴瘤样病变的重要线索。
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