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Primary Cutaneous γδ T-Cell Lymphoma and Hemophagocytic Syndrome

Moshrefi, Shawn BSc; Saad, Ahmad MD; Minoo, Parham MD; Wang, Huan-You MD, PhD; Tenenhaus, Mayer MD

doi: 10.1097/SAP.0000000000000165
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Primary cutaneous γδ T-cell lymphoma and hemophagocytic syndrome (HPS) is a very rare disease process with only 41 cases of this type of lymphoma published to date. We report the case of a 56-year-old woman who developed high-grade fevers and multiple nonhealing bilateral lower extremity ulcers associated with a recent diagnosis of Ackerman syndrome. Multiple lesion biopsies yielded a differential diagnosis including sarcoidosis and lupus panniculitis. Each biopsy site developed into a poorly healing wound. After extensive rheumatologic and dermatologic workup, failed courses of hyperbaric oxygen and corticosteroids, and the development of worsening fevers, the diagnosis of γδ T-cell lymphoma was made. The objective of this article was to illustrate a very rare case of a rapidly progressing cutaneous lymphoma while stressing the importance of a surgical approach to nonhealing wounds and biopsy in chronic wounds.

An aggressive multidisciplinary approach including infectious disease, hematology/oncology, nephrology, plastic surgery, and interventional pulmonology was used. Medical approaches included several courses of corticosteroids and antineoplastics. Our surgical approach included numerous excision and debridements of her nonhealing ulcers with removal of necrotic tissue. It also included placement of cadaveric epidermal graft, and bovine tendon collagen cross-linked with glycosaminoglycans with a silicone matrix bilayer (Integra, Plainsboro, NJ) combined with multiple local myocutaneous advancement flaps. This approach demonstrated clinical improvement in wound healing. Pathological examination of several lesions included immunohistochemistry staining, flow cytometry, and molecular studies.

The diagnosis of primary cutaneous γδ T-cell lymphoma was made based on a pathology specimen. Subsequent imaging showed numerous pulmonary nodules later determined to be pulmonary Aspergillosis. Additionally, she developed a constellation of clinical and laboratory features defined as the HPS. Despite medical therapy and improved wound healing, she died to seizures that left her comatose, thought to be secondary to central nervous system advancement of her HPS. The patient eventually died after care was withdrawn.

Cutaneous forms of lymphoma must be considered in the differential diagnosis of atypical cutaneous lesions or nonhealing wounds. There should be no delay in performing a skin biopsy to obtain a tissue diagnosis. A surgical approach to wounds clearly demonstrates improved wound healing.

From the Department of Plastic Surgery, University of California, San Diego, San Diego, CA.

Received August 12, 2013, and accepted for publication, after revision, January 22, 2014.

Conflicts of interest and sources of funding: none declared.

Reprints: Shawn Moshrefi, BSc, Department of Plastic Surgery, University of California, San Diego, 200 West Arbor Dr, San Diego, CA. E-mail: shmoshre@ucsd.edu.

Presented at the 63rd Annual Meeting of the CSPS and is being submitted for inclusion in the dedicated CSPS-Annals April 2014 issue highlighting this meeting.

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