Cutaneous presentation of enteropathy-associated T-cell lymphoma masquerading as a DUSP22-rearranged CD30+ lymphoproliferation

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 c...

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Bibliographic Details
Published in:Virchows Archiv : an international journal of pathology 2022-10, Vol.481 (4), p.653-657
Main Authors: Bisig, Bettina, Cairoli, Anne, Gaide, Olivier, Somja, Joan, Bregnard, Cloé, Gaulard, Philippe, Xerri, Luc, Lefort, Karine, Missiaglia, Edoardo, Gilliet, Michel, Hohl, Daniel, Guenova, Emmanuella, de Leval, Laurence
Format: Article
Language:English
Subjects:
Autoimmune diseases
Brief Report
CD30 antigen
Celiac disease
Differential diagnosis
Immunoproliferative diseases
Intestine
Janus kinase
Lymphatic diseases
Lymphocytes
Lymphocytes T
Lymphoma
Medicine
Medicine & Public Health
Mutation
Pathology
Stat3 protein
T-cell lymphoma
Tumors
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Summary: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.
ISSN:0945-6317
1432-2307
DOI:10.1007/s00428-022-03309-4