1/13. A case of natural killer/T cell lymphoma of the subcutis resembling subcutaneous panniculitis-like T cell lymphoma.A case of nasal type natural killer (NK)/T cell lymphoma of the subcutis showing clinical and morphological features that resemble subcutaneous panniculitis-like T cell lymphoma (SPTCL) is presented. A 73-year-old man presented with swelling of the left arm and was diagnosed with panniculitis by a dermatologist. It was concluded from a skin biopsy specimen that the patient had non-Hodgkin's lymphoma of the large cell, NK/T cell type because the neoplastic cells showed polyclonal CD3 immunoreactivity. Treatment with interferon-gamma was initiated, but the patient died of disseminated intravascular coagulation and multiple organ failure 2 months after the initial symptoms appeared. However, involvement of additional organs by the lymphoma was not apparent clinically. An autopsy was not performed. A routinely stained section of the biopsy skin specimen revealed massive necrosis of the subcutaneous fat, karyorrhexis admixed with reactive histiocytes, and large atypical lymphoid cells. Immunoreactivity for polyclonal CD3 was present in the perinuclear region, but absent in the neoplastic cell membranes. CD56, CD45RO (UCHL-1), CD43 (MT1), CD45 (leukocyte common antigen), and the cytotoxic molecules perforin, granzyme B and TIA-1 were positive, but CD20 (L26), CD4, CD8, and betaF1 were negative. Epstein-Barr virus (EBV) mRNA was detected in the nuclei of neoplastic cells by in situ hybridization. Subcutaneous panniculitis-like T cell lymphoma is reported to be an EBV-negative, clonal T cell neoplasm. Although this case showed clinical and morphological features that resembled SPTCL, perinuclear polyclonal CD3 staining and membranous CD56 reactivity seen in neoplastic cells were suggestive of NK cells. Furthermore, the neoplastic cells were positive for EBV. This case is considered to be a NK/T cell lymphoma of the subcutis resembling SPTCL. It is believed that it is important to recognize such a tumor because patients may undergo a fulminant clinical course, despite the tumor being localized in the subcutaneous adipose tissue.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/13. Subcutaneous panniculitis by Epstein-Barr virus-infected natural killer (NK) cell proliferation terminating in aggressive subcutaneous NK cell lymphoma.We describe here a case involving a patient presenting initially with subcutaneous panniculitis, which developed after 12 years into aggressive subcutaneous natural killer (NK) cell lymphoma with peripheral blood involvement and hemophagocytosis. The surface marker of lymphoid cells in peripheral blood was CD2 3-7 8-16 56 . skin biopsies were taken in May 1986 and June 1998. The initial biopsy revealed a diffuse proliferation of atypical lymphoid cells in the subcutaneous tissue with panniculitis, while the second biopsy revealed the presence of large lymphoid cells in the subcutaneous tissue with necrotic changes, consistent with a diagnosis of malignant lymphoma (diffuse pleomorphic type). The lymphoid cells from these two specimens were positive for CD56 and such cytotoxic molecules as T-cell intracellular antigen-1 (TIA-1), granzyme B, and, interestingly, also positive for Epstein-Barr (EB) virus by in situ hybridization. This suggests that chronic EB virus infections play an important role in the early stages of tumorigenesis and in the progression of NK cell lymphoproliferative disorders.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/13. An ultrastructural study of cutaneous panniculitis-like T-cell lymphoma: cytoplasmic granules and active cellular and cell-to-matrix interaction mimic cytotoxic T-cells.Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of peripheral T-cell-derived lymphoma. A 30-year-old Japanese woman presented, complaining of skin lesions on her left hip. The cellular constituents in this SPTCL were of a mixed population. Not only tumor lymphoid cells, but also many macrophages, endothelial, fibroblasts, and fat cells were seen. The tumor cells immunostained positive for CD3 and CD8, but negative for CD4. Cytotoxic injury granule-related antigens of TIA-1 and granzyme B were positive in tumor cells. CD30. CD56, EBNA-2, LMP-1, CD20cy, and CD68 were all negative in the tumor cells. An ultrastructural study revealed that the lymphoma cells showed primitive cellular contacts with the neighboring tumor cells, interacted with the short villous dendrites of the opposing macrophage and fibroblast cellular membranes, and were associated with the vascular constituents, fat cells, and the extracellular matrix. Small aggregations of the granules were frequently seen in the cytoplasm. It was speculated that the tumor cells to some extent preserve the cytotoxic T-cell structure and function, have active cellular and cell-to-matrix interaction, contain characteristic cytoplasmic granules, and reveal unique histology like panniculities.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/13. Subcutaneous panniculitis-like T-cell lymphoma: presentation of 2 cases and observations.Subcutaneous panniculitis-like T-cell lymphoma is a rare tumor of primary cutaneous origin representing far < 1% of all non-Hodgkin's lymphomas. The disease typically follows a distinctive, indolent course of recurrent, self-healing subcutaneous nodules. These nodules mimic lipomas clinically, while histologically resembling a panniculitis. Alternatively, a rapidly progressive course might be seen with subcutaneous nodules accompanied by constitutional symptoms and, in some cases, the development of a potentially fatal hemophagocytic syndrome with significant cytopenia. This tumor is widely regarded as a tumor of CD8 cytotoxic T cells with the presence of cytotoxic proteins, T-cell-restricted intracellular antigen, and granzyme B commonly demonstrated. A number of modalities have been reported in the treatment of this tumor, with varying degrees of success. In this report, we present 2 cases of subcutaneous panniculitis-like T-cell lymphoma with variable clinical courses. We also review the literature of this unusual lymphoma.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/13. Cytotoxic gamma/delta subcutaneous panniculitis-like T-cell lymphoma: report of a case with pulmonary involvement unresponsive to therapy.Peripheral subcutaneous panniculitis-like T-cell lymphoma (PSPTCL) is a rare form of cutaneous lymphoma recently proposed as a distinct clinicopathological entity. It usually presents with multiple indurated subcutaneous plaques or tumours, most commonly located on the extremities and trunk and clinically mimicking lobular panniculitis. Associated constitutional symptoms due to haemophagocytic syndrome may advance or, more often, complicate the clinical course in about 40-70% of cases. Finding of TIA-1 and perforin cytolytic granules in atypical pleomorphic lymphocytes suggests PSPTCL origin from granular cells of T-cell or natural killer cell phenotype. cells have a CD3 CD4 CD8- or CD3 CD4- CD8 T-cell phenotype. Moreover, these lymphomas can express natural killer cell associated antigens, such as CD56, especially in gamma/delta variants. PSPTCL following an indolent clinical course with recurrent self-healing lesions have been described. The prognosis of most PSPTCL is poor even when treated with aggressive chemotherapy. This paper reports a case of PCTCL in a young woman with T-cytotoxic differentiation, with rapid progression unresponsive to several treatments.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/13. Successful treatment of a patient with subcutaneous panniculitis-like T-cell lymphoma with high-dose chemotherapy and total body irradiation.A 24-yr-old man was referred for fever, right cheek swelling, subcutaneous tumor and liver dysfunction. physical examination showed an elastic hard subcutaneous tumor on the right cheek, left axillary lymph node swelling and multiple small subcutaneous tumors in the trunk. Laboratory examinations showed elevated levels of transaminase, soluble interleukin-2 receptor and ferritin. biopsy of the subcutaneous tumor showed proliferation of medium-sized cells with abundant clear cytoplasm and hyperchromatic nuclei among the subcutaneous fat tissues. These cells showed CD3 , CD4-, CD8 , CD56- and CD20- phenotype and possessed cytotoxic molecules such as granzyme B and T-cell intracellular antigen-1. bone marrow aspiration showed proliferation of small numbers of abnormal lymphocytes with severe hemophagocytosis. He was thus diagnosed as having subcutaneous panniculitis-like T-cell lymphoma (SPTCL) and treated with dose-escalated CHOP regimen. After three courses of the chemotherapy, he was further treated with high-dose chemotherapy and total body irradiation (TBI) with autologous peripheral blood stem cell rescue. Thereafter, he has been in remission for more than 2 yr. We consider that SPTCL with hemophagocytosis is an extremely aggressive disease, and high-dose chemotherapy and TBI should be included for the choice of the treatment.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/13. Nasal-type natural killer cell lymphoma preceded by benign panniculitis arising in an asymptomatic HTLV-1 carrier.We report a case of an Epstein-Barr virus (EBV)-associated nasal-type natural killer cell lymphoma (NKCL) preceded by benign panniculitis, which arose in a 48-year-old woman with an asymptomatic human T-cell leukemia/lymphoma virus type-1 (HTLV-1) infection. A biopsy of the initial panniculitis lesion demonstrated lobular panniculitis with a germinal center composed of benign mononuclear cells with a phenotype of CD4 CD45RO CD5sCD3 cCD3 epsilon T-cell intracellular antigen-1 (TIA-1)- and granzyme B-. One year after oral prednisolone therapy, the patient developed subcutaneous nodules composed of atypical lymphoid cells with a phenotype of CD4-CD45RO CD56 sCD3-cCD3 epsilon (TIA-1) and granzyme B . In the initial panniculitis lesion, neither EBV-encoded rna (EBER-1) nor clonal proliferation of EBV-infected cells was identified. In later lesions, however, a large number of atypical cells were positive for EBER-1, and a clonal expansion of EBV-infected cells was detected. No clonal rearrangement of T-cell receptor-alpha, -beta, or -gamma genes was found in either specimen. This patient was an asymptomatic carrier of human T-cell leukemia/lymphoma virus type-1 (HTLV-1) without clonal integration of proviral HTLV-1 in neither the peripheral blood nor the skin lesions. These observations suggest that EBV-associated NKCL occurred subsequently in the clinical course of benign panniculitis under the influence of immunosuppression caused by prednisolone treatment and HTLV-1 infection.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/13. A case of cytophagic histiocytic panniculitis with sicca symptoms and lupus nephritis.A 48-year-old Japanese woman presented with many subcutaneous nodules. The skin was purplish in color and tender; the nodules were scattered over the entire surface. Histological findings of biopsy specimens from the nodules indicated septal panniculitis comprised of histiocyte and/or macrophage infiltrates, often with erythro- and/or leukophagocytosis. Phagocytic cells were OKM1 (CD11b), MT1 (CD43), LeuM3 (CD14), and histiocyte antigen positive, indicating the presence of histiocytes and/or macrophages. The patient had sicca symptoms, positive homogenous, speckled pattern ANA (x320), and diffuse proliferative lupus nephritis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/13. Histiocytic lymphophagocytic panniculitis (Rosai-Dorfman disease): a case report.A 68-year-old woman presented with a symmetrical enlargement of the face and submandibular area associated with essential thrombocytosis. biopsy of the lesions revealed a septal and lobular panniculitis disclosing a lymphohistiocytic and plasma cell proliferation with lymphophagocytosis by histiocytes, compatible with Rosai-Dorfman disease. The lesions regressed spontaneously without therapy over a period of 4 months. diagnosis was confirmed by electron-microscopic examination and immunocytochemical presence of S-100 antigen within the histiocytic cells. A 2-year follow-up did not demonstrate any evidence of recurrence.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/13. Pseudosclerodermatous panniculitis after irradiation and bronchiolitis obliterans organizing pneumonia: simultaneous onset suggesting a common origin.Due to the technical evolution of radiation therapy of breast carcinoma, new manifestations occur as side effects. Newly described unexpected cutaneous events are morphea of the breast and pseudosclerodermatous panniculitis after irradiation (PPAI). They appear as specific consequences of megavoltage radiation. radiotherapy-induced bronchiolitis obliterans organizing pneumonia (R-BOOP) is one of the pulmonary manifestations due to recent advances in radiotherapy of breast carcinoma. We report a case of PPAI and R-BOOP of simultaneous onset in a 60-year-old woman 6 months after megavoltage radiation therapy for breast carcinoma. We also noted an improvement of both unexpected reactions with systemic corticosteroid therapy and a common relapse when tapering the treatment. Only eight cases of PPAI have previously been reported. We believe that this entity is often underdiagnosed. The symmetrical onset and parallel evolution suggest a common origin for PPAI and BOOP in our case: the megavoltage radiation therapy. The pathogeny of R-BOOP is not direct toxicity of irradiation because untreated areas can also be affected. Pathogenesis of PPAI remains unknown, but a simultaneous onset with R-BOOP suggests a similar mechanism. While a cumulative dose of irradiation does not seem to be determinant an immune reaction to neoantigens induced by radiation could be an hypothesis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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