Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The cytotoxicity of DNA-specific autoantibodies from sera of patients with systemic lupus erythematosis (SLE) and with lymphoproliferative diseases, and from blood of healthy donors was examined on tumor-cell lines L929 and HL-60. DNA-binding IgG fractions from SLE and chronic lymphocytic leukemia (CLL) sera were cytotoxic at concentrations of up to 10(-10) M. No detectable changes in cell viability were observed after incubation with antibodies devoid of DNA-binding activity and DNA-specific antibodies isolated from blood of healthy donors and patients with T-cell lymphoma, B-cell lymphosarcoma, and acute B-cell leukemia. There was good correlation between the cytotoxic activity and DNA-hydrolyzing activity of anti-DNA antibodies. The cytotoxic effect of DNA-binding antibodies presumably was complement-independent, because it was attributed only to the Fab fragment. The cytotoxic effect was completely inhibited by preincubation with double-stranded DNA (dsDNA). Both the cytotoxic effect and the DNA-hydrolyzing activity of anti-DNA antibodies were significantly increased in the antibody fraction that displayed cross-reactivity with nuclear matrix proteins. Possible mechanisms for the formation and pathogenicity of cytotoxic anti-DNA antibodies are discussed in this article.
 ...
PMID:Autoantibodies to nuclear antigens: correlation between cytotoxicity and DNA-hydrolyzing activity. 1082 65

Mycosis fungoides is a form of cutaneous malignant T-cell lymphoma initially confined to the skin. A range of ophthalmic manifestations has been described; these usually appear in advanced disease. Lid involvement is most common and ranges from ectropion, usually cicatricial, to diffuse thickening and placoid tumours. A case of a 72-year-old man presenting with progressive, full thickness, lower eyelid ulceration is presented. The patient had a long history of placoid skin lesions, previously diagnosed as discoid lupus. Repeat biopsies of these lesions including the eyelid eventually revealed mycosis fungoides. The ulceration progressed to destroy most of the lower lid before the correct diagnosis was made. This type of eyelid involvement has not previously been reported in mycosis fungoides. This case also highlights the diagnostic difficulties encountered in the early phases of mycosis fungoides. If suspected, multiple biopsies are essential to establish the diagnosis.
 ...
PMID:Mycosis fungoides causing severe lower eyelid ulceration. 1221 65

We report on the characteristics of 21 patients with hepatosplenic gammadelta T-cell lymphoma (HSgammadeltaTCL), an entity recognized since 1994 in the Revised European American Lymphoma (REAL) classification. Median age was 34 years. Patients had splenomegaly (n = 21), hepatomegaly (n = 15), and thrombocytopenia (n = 20). Histopathologic findings were homogeneous and showed the presence of medium-sized lymphoma cells within the sinusoids of splenic red pulp, liver, and bone marrow. Marrow involvement was usually mild but could be demonstrated by phenotyping in all patients. Cells were CD3+CD5-, expressed the gammadelta T-cell receptor, and had a nonactivated cytotoxic cell phenotype (TIA-1+, granzyme B-). Most patients were CD4-/CD8- (16 of 18); CD56+ (15 of 18), expressed the Vdelta1epitope (Vd1+/Vd2-/Vd3-) (9 of 12); and were negative for Epstein-Barr virus (EBV) (18 of 20). Isochromosome arm 7q was documented in 9 of 13 patients. Eight patients had previously undergone kidney transplantation or had a history of systemic lupus, Hodgkin disease, or malaria. Prognosis was poor; median survival time was 16 months, and all but 2 patients ultimately died despite consolidative or salvage high-dose therapy. In conclusion, HSgammadeltaTCL is a disease with distinctive clinical, histopathologic, and phenotypic characteristics. Bone marrow biopsy with combined phenotyping is sufficient for diagnosis, and splenectomy is therefore unwarranted. Current treatment modalities appear to be ineffective in most patients.
 ...
PMID:Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients. 1290 41

Lupus erythematosus (LE) panniculitis (LEP) is a form of chronic cutaneous LE most often characterized by erythematous subcutaneous nodules. The histopathology of LEP may be distinctive, allowing a diagnosis even in the absence of any other LE features. Lymphocytic infiltration of the panniculus is termed lymphocytic lobular panniculitis. This entity has been reported in both LEP and subcutaneous panniculitis-like T-cell lymphoma. We describe a 67-year-old woman who presented with multiple dermal and subcutaneous nodules on her legs and hips. Biopsy specimens of roughly 15 lesions were interpreted as being diagnostic of LEP, however, with each subsequent biopsy specimen more atypical cells were observed. The patient responded only to moderate doses of oral prednisone, and failed intralesional triamcinolone acetonide, oral immunosuppressive agents, oral antimalarial agents, and oral thalidomide. Three years after the onset of her disease, ulcerated plaques and nodules developed. At this time a T-cell receptor gene rearrangement was present and subcutaneous panniculitis-like T-cell lymphoma was diagnosed. Despite multiple doses of chemotherapy she died approximately 1 year after diagnosis. Patients with apparent LEP that have atypical lymphocytes in their biopsy specimens should be followed up closely for the development of T-cell malignancy.
 ...
PMID:Subcutaneous panniculitis-like T-cell lymphoma with vacuolar interface dermatitis resembling lupus erythematosus panniculitis. 1498 94

Lupus panniculitis is characterized by a T-cell lymphocyte infiltration of the fat, with fibrosing and cicatricial progression. The lesions are predominantly found on the head and the upper part of the body; they are more frequent in young women. Diagnosis is based on the integration of the clinical and histological data, which renders a deep cutaneous biopsy indispensable. Histopathology is the key to the differential diagnosis, which essentially includes subcutaneous T-cell lymphoma. The lupus panniculitis lesions can be isolated or, more often, integrated within a known lupus disease, usually of good prognosis. Treatment relies above all on synthetic anti-malarials, occasionally associated with local or general corticosteroids.
 ...
PMID:[Lupus panniculitis]. 1579 39

We report a 48-year-old man who presented with ulcerated plaques and nodules of the lower extremities. Skin biopsies revealed a dense lymphocytic infiltrate involving the dermis and the subcutis in a lobular and septal pattern. No overt cytological atypia was present. Notably, several features resembling lupus erythematosus were present, including vacuolar interface change and abundant dermal mucin deposition. The patient developed pulmonary nodules, and a lung biopsy showed a perivascular and interstitial lymphoid infiltrate without overt atypia. The cutaneous and pulmonary lymphoid infiltrates showed similar immunohistochemical profiles: CD3(+) CD4(-) CD8(+/-) CD56(+). Monoclonal rearrangements of the T-cell receptor gamma gene with similar migration patterns were identified from both locations. The patient developed fatal hemophagocytic syndrome, involving liver, spleen, lymph nodes, and bone marrow. This case is one amongst the rare reports of subcutaneous panniculitis-like T-cell lymphoma with systemic involvement.
 ...
PMID:Fatal subcutaneous panniculitis-like T-cell lymphoma with interface change and dermal mucin, a dead ringer for lupus erythematosus. 1581 Nov 22

We report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL), associated with macrophage activation syndrome, mimicking a lupus erythematosus panniculitis (LEP). A 29-year-old woman presented with high fever, general malaise, nausea, vomiting, and subcutaneous nodules and ulcerating lesions located on the lower extremities. The histopathology showed an infiltration of the panniculus, mostly involving fat, and periadnexial and perivascular structures consistent with lymphocytic lobular panniculitis (LLP). LLP is a shared feature of LEP and SPTCL. The immunophenotyping of the cell infiltrate was crucial for a correct diagnosis.
 ...
PMID:Subcutaneous panniculitis-like T-cell lymphoma misdiagnosed as lupus erythematosus panniculitis. 1640 99

In the newly revised World Health Organization (WHO)-European Organization for Research and Treatment of Cancer (EORTC) consensus classification for cutaneous lymphomas, cutaneous gamma/delta T-cell lymphoma (CGD-TCL) has been included as a provisional entity. This type of lymphomas, when involving the subcutaneous fat, can mimic both clinically and histologically other more indolent conditions, such as subcutaneous panniculitic T-cell lymphomas (SPTCL) and lupus erythematosus profundus (LEP), and multiple biopsies may be needed to obtain a correct diagnosis. A good correlation of the clinical data with the histopathology and immunohistochemistry are required for diagnosis. Herein, we describe a patient whose initial histopathologic findings ressembled LEP but presented an aggressive clinical course. A new biopsy was performed during the follow-up, and a final diagnosis of CGD-TCL was made.
 ...
PMID:Cutaneous gamma/delta T-cell lymphoma: a histopathologic mimicker of lupus erythematosus profundus (lupus panniculitis). 1736 13

Subcutaneous panniculitis-like T cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma classified in the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification as a unique extranodal lymphoma with characteristic by T cell receptor (TCR) gene rearrangement. We report here a case of SPTCL in a 22 year-old woman who had presented with variably sized multiple nodules on both her legs. Initial differential diagnoses considered were panniculitis and lupus panniculitis. The histopathology showed a predominantly subcutaneous lobular infiltrate with atypical lymphocytes, karyorrhexis and rimming of adipocytes by lymphoid cells. Immunohistochemistry showed CD4-, CD8+, CD56- T-cell phenotype. Although TCR rearrangement studies were not done, the above T-cell phenotype and sparing of epidermis and dermis suggested the possibility of an SPTCL alpha/beta type. The patient received five cycles of a cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) regimen which resulted in the regression in her skin lesions and constitutional symptoms.
 ...
PMID:Subcutaneous panniculitis-like T-cell cutaneous lymphoma. 1838 78

Lupus erythematosus panniculitis is an uncommon variant of lupus erythematosus characterized by a specific involvement of the subcutaneous fat. It is a panniculitis with peculiar clinical features and histopathologically characterized by a mostly lobular panniculitis. It may appear in patients with discoid lupus erythematosus and systemic lupus erythematosus, but also as the unique manifestation of lupus erythematosus, and in the latter cases the diagnosis may be problematic. Histopathologic differential diagnosis with subcutaneous panniculitis-like T-cell lymphoma may also be extremely difficult. This article reviews the salient clinicopathologic features and treatment of lupus erythematosus panniculitis, with special emphasis on the histopathologic features.
 ...
PMID:Lupus erythematosus panniculitis. 1879 77


1 2 3 Next >>