Cutaneous γδ T-cell Lymphomas: A Spectrum of Presentations With Overlap With Other Cytotoxic Lymphomas.

Nov 2012

Guitart J, Weisenburger DD, Subtil A, Kim E, Wood G, Duvic M, Olsen E, Junkins-Hopkins J, Rosen S, Sundram U, Ivan D,Selim MA, Pincus L, Deonizio JM, Kwasny M, Kim YH.

Source

*Departments of Dermatology and Pathology ††Department of Hematology and Oncology, Northwestern University Feinberg Medical School, Chicago, IL †Department of Pathology, University of Nebraska Medical Center, NE ‡Department of Dermatology, Yale University, New Haven, CT §Department of Dermatology, University of Pennsylvania, Philadelphia, PA ∥Department of Dermatology, University of Wisconsin, WI ¶Department of Dermatology, MD Anderson, Houston, TX #Departments of Dermatology and Pathology, Duke University, Durham, NC **Department of Dermatology, Johns Hopkins University, Baltimore, MA ‡‡Department of Pathology, Stanford University, Stanford §§Department of Dermatology, University of California in San Francisco, San Francisco, CA ¶¶Department of Preventive Medicine, Northwestern University Feinberg Medical School, Chicago, IL.

Abstract

We reviewed our multicenter experience with gamma-delta (γδ) T-cell lymphomas first presenting in the skin. Fifty-three subjects with a median age of 61 years (range, 25 to 91 y) were diagnosed with this disorder. The median duration of the skin lesions at presentation was 1.25 years (range, 1 mo to 20 y). The most common presentation was deep plaques (38 cases) often resembling a panniculitis, followed by patches resembling psoriasis or mycosis fungoides (10 cases). These lesions tended to ulcerate overtime (27 cases). Single lesions or localized areas of involvement resembling cellulitis or pyoderma were reported in 8 cases. The most common anatomic site of involvement was the legs (40 cases), followed by the torso (30 cases) and arms (28 cases). Constitutional symptoms were reported in 54% (25/46) of the patients, including some with limited skin involvement. Significant comorbidities included autoimmunity (12 cases), other lymphoproliferative disorders (5 cases), internal carcinomas (4 cases), and viral hepatitis (2 cases). Lymphadenopathy (3/42 cases) and bone marrow involvement (5/28 cases) were uncommon, but serum lactose dehydrogenase (LDH) was elevated in 55% (22/39) of the patients. Abnormal positron emission tomography and/or computed tomography scans in 20/37 subjects mostly highlighted soft tissue or lymph nodes. Disease progression was associated with extensive ulcerated lesions resulting in 27 deaths including complications of hemophagocytic syndrome (4) and cerebral nervous system involvement (3). Median survival time from diagnosis was 31 months. Skin biopsies varied from a pagetoid pattern to purely dermal or panniculitic infiltrates composed of intermediate-sized lymphocytes with tissue evidence of cytotoxicity. The most common immunophenotype was CD3/CD4/CD5/CD8/BF1/γ-M1/TIA-1/granzyme-B/CD45RA/CD7, and 4 cases were Epstein-Barr virus positive. This is the largest study to date of cutaneous γδ T-cell lymphomas and demonstrates a variety of clinical and pathologic presentations with a predictable poor outcome.

Lippincott Williams & Wilkins