Transformation of Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma to Interdigitating Dendritic Cell Sarcoma

被引：71

作者：

Fraser, Cory R. ^{[1
]}

Wang, Wei ^{[3
]}

Gomez, Mario ^{[4
]}

Zhang, Taotao ^{[1
]}

Mathew, Susan ^{[1
]}

Furman, Richard R. ^{[2
]}

Knowles, Daniel M. ^{[1
]}

Orazi, Attilio ^{[1
]}

Tam, Wayne ^{[1
]}

机构：

[1] Weill Cornell Med Coll, Div Hematopathol, Dept Pathol & Lab Med, New York, NY 10021 USA

[2] Weill Cornell Med Coll, Div Hematol Oncol, Dept Med, New York, NY 10021 USA

[3] Cornell Univ, Microarray Core Facil, Ithaca, NY USA

[4] New York Coll Osteopath Med, New York, NY USA

来源：

AMERICAN JOURNAL OF CLINICAL PATHOLOGY | 2009年 / 132卷 / 06期

关键词：

Chronic lymphocytic leukemia; Interdigitating dendritic cell sarcoma; Transformation; Transdifferentiation; NF-KAPPA-B; TRANSCRIPTION FACTORS; C/EBP; HISTIOCYTOSIS; EXPRESSION; PU.1;

D O I：

10.1309/AJCPWQ0I0DGXBMHO

中图分类号：

R36 [病理学];

学科分类号：

100104 ;

摘要：

Interdigitating dendritic cell sarcoma (IDCS) is a rare tumor derived from interdigitating dendritic cells. Three cases of IDCS associated with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) have been described, but no clonal relationship between the 2 neoplasms was demonstrated. We present a detailed case analysis of a CLL/SLL with metachronous IDCS and demonstrate that these 2 neoplasms are clonally related. The IDCS and CLL cells had trisomy 12 and identical monoclonal immunoglobulin heavy chain gene rearrangements. Analysis of transcription factors with a role in myeloid differentiation demonstrated PU. I up-regulation and Cl EBP alpha down-regulation in IDCS compared with CLL. High-density array comparative genomic hybridization also identified gains in part of chromosome 16q in IDCS. Our study demonstrates for the first time clonal transformation of CLL/SLL into IDCS. This phenomenon may be triggered by alterations in lineage-determining transcription programs, which result in transdifferentiation, coupled with additional oncogenic stimuli caused by chromosomal imbalances.

引用

页码：928 / 939

页数：12

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