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

Submitted April 4, 2003
Accepted May 12, 2003
ALK activation by the CTLC-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, Genevieve Laureys, Paul Brons, Peter Vandenberghe, Frank Speleman, Marleen Praet, Chris De Wolf-Peeters, Peter Marynen, and Iwona Wlodarska*
Department of Pathology, Ghent University Hospital, Ghent, Belgium
Department of Human Genetics and Flanders Interuniversity Institute for Biotechnology, Catholic University of Leuven, Leuven, Belgium
Department of Pathology, University Medical Center Nijmegen, Nijmegen, The Netherlands
Department of Clinical Chemistry, Microbiology and Immunology, Ghent University Hospital, Ghent, Belgium
Department of Molecular Diagnostics, Ghent University Hospital, Ghent, Belgium
Department of Human Genetics, University Medical Center Nijmegen, Nijmegen, The Netherlands
Center of Medical Genetics, Ghent University Hospital, Ghent, Belgium
Department of Pediatric Oncology, Ghent University Hospital, Ghent, Belgium
Department of Pediatric Oncology, University Medical Center Nijmegen, Nijmegen, The Netherlands
Division for Morphology and Molecular Pathology, Catholic University of Leuven, Leuven, Belgium
* Corresponding author; email: Iwona.Wlodarska{at}uz.kuleuven.ac.be.
We present three cases of large B-cell lymphoma (LBCL) with a granular cytoplasmic staining for ALK kinase. All of them 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 ALCL, the B-cell phenotype only became evident at recurrence. 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.

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