[en] DUSP22 gene rearrangements are recurrent in systemic and cutaneous ALK-negative anaplastic large cell lymphomas, rarely encountered in other cutaneous CD30+ lymphoproliferations, and typically absent in other peripheral T-cell lymphomas. We report the case of a 51-year-old woman, with longstanding celiac disease and a rapidly enlarging leg ulcer, due to a DUSP22-rearranged CD30+ T-cell lymphoproliferation. Subsequent history revealed an intestinal enteropathy-associated T-cell lymphoma (EATL). Identical monoclonal TR gene rearrangements and mutations in STAT3 and JAK1 typical of EATL were present in the cutaneous and intestinal lesions. No DUSP22 rearrangement was detected in the patient's intestinal tumour, nor in 15 additional EATLs tested. These findings indicate that DUSP22 rearrangements are not entirely specific of ALCLs, may rarely occur as a secondary aberration in EATL, and expand the differential diagnosis of DUSP22-rearranged cutaneous CD30+ lymphoproliferative disorders.
Disciplines :
Hematology
Author, co-author :
Bisig, Bettina ; Institute of Pathology, Department of Laboratory Medicine and Pathology, Lausanne University Hospital and Lausanne University, Rue du Bugnon 25, CH-1011, Lausanne, Switzerland
Cairoli, Anne; Service of Haematology, Department of Oncology, Lausanne University Hospital and Lausanne University, Lausanne, Switzerland
Gaide, Olivier; Division of Dermatology, Department of Medicine, Lausanne University Hospital and Lausanne University, Lausanne, Switzerland
Somja, Joan ; Université de Liège - ULiège > Département des sciences biomédicales et précliniques
Bregnard, Cloé; Institute of Pathology, Department of Laboratory Medicine and Pathology, Lausanne University Hospital and Lausanne University, Rue du Bugnon 25, CH-1011, Lausanne, Switzerland
Gaulard, Philippe; Department of Pathology, Henri Mondor Hospital, AP-HP, INSERM U955, Mondor Institute for Biomedical Research (IMRB) and Paris Est Creteil University, Créteil, France
Xerri, Luc; Department of Pathology and Tumor Immunology, Institut Paoli-Calmettes, Research Center in Cancer of Marseille, INSERM U1068, CNRS UMR7258, Aix-Marseille University, UM105, Marseille, France
Lefort, Karine; Institute of Pathology, Department of Laboratory Medicine and Pathology, Lausanne University Hospital and Lausanne University, Rue du Bugnon 25, CH-1011, Lausanne, Switzerland
Missiaglia, Edoardo; Institute of Pathology, Department of Laboratory Medicine and Pathology, Lausanne University Hospital and Lausanne University, Rue du Bugnon 25, CH-1011, Lausanne, Switzerland
Gilliet, Michel; Division of Dermatology, Department of Medicine, Lausanne University Hospital and Lausanne University, Lausanne, Switzerland
Hohl, Daniel; Division of Dermatology, Department of Medicine, Lausanne University Hospital and Lausanne University, Lausanne, Switzerland
Guenova, Emmanuella; Division of Dermatology, Department of Medicine, Lausanne University Hospital and Lausanne University, Lausanne, Switzerland
de Leval, Laurence ; Institute of Pathology, Department of Laboratory Medicine and Pathology, Lausanne University Hospital and Lausanne University, Rue du Bugnon 25, CH-1011, Lausanne, Switzerland. Laurence.DeLeval@chuv.ch
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