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Prepublished online as a Blood First Edition Paper on May 15, 2003; DOI 10.1182/blood-2003-04-1050.

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Blood, 1 October 2003, Vol. 102, No. 7, pp. 2638-2641

NEOPLASIA
Brief report

ALK activation by the CLTC-ALK fusion is a recurrent event in large B-cell lymphoma

Pascale De Paepe, Mathijs Baens, Han van Krieken, Bruno Verhasselt, Michel Stul, Annet Simons, Bruce Poppe, Geneviève Laureys, Paul Brons, Peter Vandenberghe, Frank Speleman, Marleen Praet, Chris De Wolf-Peeters, Peter Marynen, and Iwona Wlodarska

From the Departments of Pathology, Clinical Chemistry, Microbiology and Immunology, and Molecular Diagnostics, Center of Medical Genetics and Pediatric Oncology, Ghent University Hospital, Belgium; the Department of Human Genetics and Flanders Interuniversity Institute for Biotechnology, Division for Morphology and Molecular Pathology, Catholic University of Leuven, Belgium; and the Departments of Pathology, Human Genetics, and Pediatric Oncology, University Medical Center Nijmegen, the Netherlands.

We present 3 cases of large B-cell lymphoma (LBCL) with a granular cytoplasmic staining for anaplastic lymphoma kinase (ALK). All of the cases showed striking similarities in morphology and immunohistochemical profile characterized by a massive monomorphic proliferation of CD20-/CD138+ plasmablast-like cells. In one of the cases, initially diagnosed as a null-type anaplastic large cell lymphoma (ALCL), the B-cell phenotype became evident only at recurrence. Fluorescent in situ hybridization (FISH) and molecular studies led to the detection of a CLTC-ALK rearrangement in all 3 cases, without any evidence of full-length ALK receptor expression. The associated t(2;17)(p23;q23) was demonstrated in the karyotype of 2 cases. Although a similar CLTC-ALK aberration was previously identified in ALK-positive T-/null cell ALCL and inflammatory myofibroblastic tumor, its association with ALK-positive LBCL seems to be specific and intriguing. (Blood. 2003;102:2638-2641)


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