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A woman in her 50s with a history of nasal-type natural killer (NK)/T-cell lymphoma (diagnosed 2 years prior; treated with pembrolizumab and methotrexate, ifosfamide, etoposide, dexamethasone, and pegaspargase) and chronic pulmonary coccidiomycosis being treated with fluconazole was admitted for fever, hypotension, and rash. Three months prior, pembrolizumab treatment was stopped because of a widespread morbilliform reaction, which had resolved completely 6 to 8 weeks before admission. The new widespread, asymptomatic skin eruption had been ongoing for 1 to 2 weeks and appeared different from her pembrolizumab reaction.
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B. Intravascular involvement by NK/T-cell lymphoma
In addition to large CD3+/CD56+ perivascular lymphocytes in the dermis, there were vessels in the subcutaneous fat with intravascular lymphocytes, with a CD3+/CD56+ immunophenotype. A diagnosis of intravascular NK/T-cell lymphoma, nasal type, was made; an Epstein-Barr virus–encoded RNA in situ hybridization study had been performed on a prior biopsy, and the result was positive. Results of CD30 immunostaining were negative. Patients with lymphoma may experience fevers owing to common or atypical infections, drugs, or as a direct consequence of lymphoma. Accordingly, a careful drug review and an extensive infectious workup were undertaken. Special stains (Fite, PAS-D, Brown-Brenn) and tissue cultures were performed and results were negative, as the clinical presentation was of fever of unknown origin.
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Corresponding Author: Ayan Kusari, MD, Department of Dermatology, University of California, San Francisco School of Medicine, 1701 Divisadero St, 4th Floor, San Francisco, CA 94115 (firstname.lastname@example.org).
Published Online: October 13, 2021. doi:10.1001/jamadermatol.2021.4068
Conflict of Interest Disclosures: Dr Haemel reported personal fees from CSL Behring for serving on a data and safety monitoring board and from Guidepoint Global for serving as a consultant outside the submitted work. No other disclosures were reported.
Additional Contributions: We thank the patient for granting permission to publish this information. We also thank Ritesh Agnihothri, MD, for providing the clinical image.
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