Fatal subcutaneous panniculitis-like T-cell lymphoma gamma/delta subtype (cutaneous gamma/delta T-cell lymphoma): report of a case and review of the literature. 2008

Grace F Kao, and Brooke Resh, and Christine McMahon, and Ivana Gojo, and Chen-Chih Sun, and Daniel Phillips, and Xianfeng Frank Zhao
Department of Pathology & Laboratory Medicine, Baltimore Veterans Affairs Medical Center, Baltimore, Maryland 21201-1524, USA. grace.kao@va.gov

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a distinct type of peripheral T-cell lymphoma, which has gained recognition over the past decade. The disease presents complex clinical, pathologic, and immunohistochemical features, which warrant awareness by dermatologists, dermatopathologists, hematopathologists, hematologists, oncologists, and internists. SPTCL was initially included as a provisional entity in the Revised European-American Lymphoma classification, followed by the European Organization for Research and Treatment of Cancer classification as a primary cutaneous lymphoma, and subsequently as a distinct entity by the World Health Organization classification. It is known that patients diagnosed with SPTCL usually respond poorly to therapy, and the tumor progresses aggressively. Data from recent studies in a series of cases of SPTCL by the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Group have further identified SPTCL as a heterogeneous disease entity, which comprises an alpha/beta subtype (SPTCL-AB) and a gamma/delta subtype (SPTCL-GD). The latter has recently been included in the entity of "cutaneous gamma/delta T-cell lymphoma" by the World Health Organization, Pathology and Genetics of Skin Tumours. The clinical, histologic, and immunophenotypic data, treatment, and prognosis, appear different in the 2 subtypes of SPTCL. We report a case of fatal SPTCL-GD (cutaneous gamma/delta T-cell lymphoma), with detailed clinicopathologic features, immunohistochemical studies, treatment, and clinical course. In view of its aggressive behavior, identification of this disease is critical for proper management and treatment.

UI MeSH Term Description Entries
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012878 Skin Neoplasms Tumors or cancer of the SKIN. Cancer of Skin,Skin Cancer,Cancer of the Skin,Neoplasms, Skin,Cancer, Skin,Cancers, Skin,Neoplasm, Skin,Skin Cancers,Skin Neoplasm
D013601 T-Lymphocytes Lymphocytes responsible for cell-mediated immunity. Two types have been identified - cytotoxic (T-LYMPHOCYTES, CYTOTOXIC) and helper T-lymphocytes (T-LYMPHOCYTES, HELPER-INDUCER). They are formed when lymphocytes circulate through the THYMUS GLAND and differentiate to thymocytes. When exposed to an antigen, they divide rapidly and produce large numbers of new T cells sensitized to that antigen. T Cell,T Lymphocyte,T-Cells,Thymus-Dependent Lymphocytes,Cell, T,Cells, T,Lymphocyte, T,Lymphocyte, Thymus-Dependent,Lymphocytes, T,Lymphocytes, Thymus-Dependent,T Cells,T Lymphocytes,T-Cell,T-Lymphocyte,Thymus Dependent Lymphocytes,Thymus-Dependent Lymphocyte
D014944 World Health Organization A specialized agency of the United Nations designed as a coordinating authority on international health work; its aim is to promote the attainment of the highest possible level of health by all peoples. Organization, World Health,WHO
D015434 Panniculitis General term for inflammation of adipose tissue, usually of the skin, characterized by reddened subcutaneous nodules. Panniculitis, Subacute Nodular Migratory,Cold Panniculitis,Cold Panniculitides,Panniculitides,Panniculitides, Cold,Panniculitis, Cold
D016410 Lymphoma, T-Cell, Cutaneous A group of lymphomas exhibiting clonal expansion of malignant T-lymphocytes arrested at varying stages of differentiation as well as malignant infiltration of the skin. MYCOSIS FUNGOIDES; SEZARY SYNDROME; LYMPHOMATOID PAPULOSIS; and PRIMARY CUTANEOUS ANAPLASTIC LARGE CELL LYMPHOMA are the best characterized of these disorders. Granulomatous Slack Skin,T-Cell Lymphoma, Cutaneous,Cutaneous T-Cell Lymphoma,Lymphoma, T Cell, Cutaneous,Cutaneous T Cell Lymphoma,Cutaneous T-Cell Lymphomas,Lymphoma, Cutaneous T-Cell,Lymphomas, Cutaneous T-Cell,Slack Skin, Granulomatous,T Cell Lymphoma, Cutaneous,T-Cell Lymphomas, Cutaneous
D016692 Receptors, Antigen, T-Cell, gamma-delta T-cell receptors composed of CD3-associated gamma and delta polypeptide chains and expressed primarily in CD4-/CD8- T-cells. The receptors appear to be preferentially located in epithelial sites and probably play a role in the recognition of bacterial antigens. The T-cell receptor gamma/delta chains are separate and not related to the gamma and delta chains which are subunits of CD3 (see ANTIGENS, CD3). Antigen Receptors, T-Cell, gamma-delta,T-Cell Receptors delta-Chain,T-Cell Receptors gamma-Chain,T-Cell Receptors, gamma-delta,TcR gamma-delta,Antigen T Cell Receptor, delta Chain,Antigen T Cell Receptor, gamma Chain,Receptors, Antigen, T Cell, gamma delta,T Cell Receptors, gamma delta,T-Cell Receptor delta-Chain,T-Cell Receptor gamma-Chain,T-Cell Receptor, gamma-delta,T Cell Receptor delta Chain,T Cell Receptor gamma Chain,T Cell Receptor, gamma delta,T Cell Receptors delta Chain,T Cell Receptors gamma Chain,TcR gamma delta,delta-Chain, T-Cell Receptor,delta-Chain, T-Cell Receptors,gamma-Chain, T-Cell Receptor,gamma-Chain, T-Cell Receptors,gamma-delta T-Cell Receptor,gamma-delta T-Cell Receptors,gamma-delta, TcR

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