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DUSP22-IRF4 rearrangement in AIDS-associated ALK-negative anaplastic large cell lymphoma.
Wang, Mike; Kibbi, Nour; Ring, Nan; Siddon, Alexa; Foss, Francine; Totonchy, Mariam.
Afiliação
  • Wang M; Dermatology, Yale University School of Medicine, New Haven, Connecticut, USA.
  • Kibbi N; Dermatology, Yale University School of Medicine, New Haven, Connecticut, USA.
  • Ring N; Dermatology, Yale University School of Medicine, New Haven, Connecticut, USA.
  • Siddon A; Laboratory Medicine and Pathology, Yale University School of Medicine, New Haven, Connecticut, USA.
  • Foss F; Dermatology, Yale University School of Medicine, New Haven, Connecticut, USA.
  • Totonchy M; Hematology, Yale University School of Medicine, New Haven, Connecticut, USA.
BMJ Case Rep ; 12(9)2019 Sep 30.
Article em En | MEDLINE | ID: mdl-31570354
ABSTRACT
Patients with AIDS have increased risk of developing lymphomas, such as anaplastic large cell lymphoma (ALCL), which generally carry a poor prognosis. The DUSP-IRF4 genetic rearrangement in ALCL confers a favourable prognosis in HIV-negative patients; it is unknown how this interacts clinically with HIV/AIDS. A man aged 53 years presented with subcutaneous nodules on the scalp and axillae, and diffuse lymphadenopathy. Biopsy of subcutaneous nodule and lymph node showed large atypical anaplastic lymphocytes which were CD30+ and anaplastic lymphoma kinase-negative, consistent with primary systemic ALCL. In addition, he was found to be HIV-positive and diagnosed with AIDS. Genetic testing of the tissue revealed a DUSP22-IRF4 rearrangement. Complete remission was achieved with HyperCVAD and subsequent brentuximab vedotin monotherapy. We report a case of AIDS-associated primary systemic ALCL with a DUSP22-IRF4 rearrangement. AIDS-associated ALCL is an aggressive lymphoma, with a poor prognosis. However, the presence of the genetic rearrangement, previously unseen in this disease, drastically altered the disease course. This case highlights the value of genetic testing and identifies DUSP22-IRF4-associated ALCL in the setting of HIV-associated lymphoproliferative disorders.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Neoplasias Cutâneas / Linfoma Relacionado a AIDS / Linfoma Anaplásico de Células Grandes / Fosfatases da Proteína Quinase Ativada por Mitógeno / Fosfatases de Especificidade Dupla / Antineoplásicos Imunológicos / Brentuximab Vedotin Tipo de estudo: Prognostic_studies / Risk_factors_studies Limite: Humans / Male / Middle aged Idioma: En Revista: BMJ Case Rep Ano de publicação: 2019 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Neoplasias Cutâneas / Linfoma Relacionado a AIDS / Linfoma Anaplásico de Células Grandes / Fosfatases da Proteína Quinase Ativada por Mitógeno / Fosfatases de Especificidade Dupla / Antineoplásicos Imunológicos / Brentuximab Vedotin Tipo de estudo: Prognostic_studies / Risk_factors_studies Limite: Humans / Male / Middle aged Idioma: En Revista: BMJ Case Rep Ano de publicação: 2019 Tipo de documento: Article