TCR-γ Expression in Primary Cutaneous T-cell Lymphomas.

Jan 2013

Rodríguez-Pinilla SM, Ortiz-Romero PL, Monsalvez V, Tomás IE, Almagro M, Sevilla A, Camacho G, Longo MI, Pulpillo A,Diaz-Pérez JA, Montes-Moreno S, Castro Y, Echevarría B, Trébol I, Gonzalez C, Sánchez L, Otín AP, Requena L,Rodríguez-Peralto JL, Cerroni L, Piris MA.

Source

*CNIO Lymphoma Group Departments of †Pathology ‡‡‡Dermatology, Fundación Jiménez Díaz Departments of ‡Dermatology §§§Pathology, Hospital Universitario 12 de Octubre §Pathology Department, Hospital de Torrejón de Ardoz Departments of **Dermatology ∥∥Pathology, Hospital Universitario Gregorio Marañón ***Dermatology Department, Hospital Universitario de Getafe, Getafe †††Immunohistochemistry Unit, Biotechnology Programme, CNIO, Madrid ∥Dermatology Department, Hospital Universitario Juan Canalejo, La Coruña ¶Dermatology Department, Hospital de San Juan, Alicante #Dermatology Department, Hospital Universitario Reina Sofía, Córdoba ††Dermatology Department, Hospital Universitario Virgen del Rocío, Sevilla §§Pathology Department, Hospital Universitario Marqués de Valdecilla, Santander ¶¶Dermatology Department, Hospital Virgen del Castillo, Murcia ##Dermatology Department, Hospital Santiago Apostol, Vitoria, Spain ‡‡UCSD Moores Cancer Center, La Joya, CA ∥∥∥Department of Dermatology, Research Unit Dermatopathology, Medical University of Graz, Graz, Austria.

Abstract

Primary cutaneous γδ T-cell lymphomas (PCGD-TCLs) are considered a subgroup of aggressive cytotoxic T-cell lymphomas (CTCLs). We have taken advantage of a new, commercially available antibody that recognizes the T-cell receptor-γ (TCR-γ) subunit of the TCR in paraffin-embedded tissue. We have analyzed a series of 146 primary cutaneous T-cell lymphomas received for consultation or a second opinion in the CNIO Pathology Department. Cases were classified according to the World Health Organization 2008 classification as mycosis fungoides (MF; n=96), PCGD-TCLs (n=5), pagetoid reticulosis (n=6), CD30 primary cutaneous anaplastic large cell lymphomas (n=5), primary cutaneous CD8 aggressive epidermotropic CTCLs (n=3), primary cutaneous CTCL, not otherwise specified (n=4), and extranodal nasal-type NK/T-cell lymphomas primarily affecting the skin or subcutaneous tissue (n=11). Sixteen cases of the newly named lymphomatoid papulosis type D (LyP-D; n=16) were also included. In those cases positive for TCR-γ, a further panel of 13 antibodies was used for analysis, including TIA-1, granzyme B, and perforin. Clinical and follow-up data were recorded in all cases. Twelve cases (8.2%) were positive for TCR-γ, including 5 PCGD-TCLs, 2 MFs, and 5 LyP-Ds. All 5 PCGD-TCL patients and 1 MF patient died of the disease, whereas the other MF patient and all those with LyP-D were alive. All cases expressed cytotoxic markers, were frequently CD3/CD8, and tended to lose CD5 and CD7 expressions. Eight of 12 and 5 of 11 cases were CD30 and CD56, respectively. Interestingly, 5/12 TCR-γ-positive cases also expressed TCR-BF1. All cases analyzed were negative for Epstein-Barr virus-encoded RNA. In conclusion, TCR-γ expression seems to be rare and is confined to cytotoxic primarycutaneous TCLs. Nevertheless, its expression is not exclusive to PCGD-TCLs, as TCR-γ protein can be found in other CTCLs. Moreover, its expression does not seem to be associated with bad prognosis by itself, as it can be found in cases with good and bad outcomes.

American Journal of Surgical Pathology

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