Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary malignant lymphoma of the liver occupying the right lobe, 14 x 9 x 7 cm in size, developed in a 30-year-old man with a 4-year history of autoimmune hemolytic anemia. The diagnosis of systemic lupus erythematosus (SLE) accompanying thrombocytopenia had been made clinically 10 months earlier. The liver biopsy specimen revealed diffuse proliferation of large lymphoma cells expressing the activated helper/inducer T-cell phenotype (LCA+, UCHL1+, OPD4+, LN3+, MT1-, L26-, MB1-, Leu M1-, Ki-1-, KP1-). The lymphoma was successfully treated by chemotherapy and irradiation. Intractable thrombocytopenia provoked fatal esophageal hemorrhage. At autopsy, no lymphomatous lesion was identified, and the hepatic right lobe contained an encapsulated necrotic lesion without any viable tumor cells. The bone marrow revealed marked hyperplasia of erythroid and megakaryocytic series. Extramedullary hematopoiesis was demonstrated in the liver, spleen and lymph nodes. This is the second case of primary hepatic T-cell lymphoma associated with SLE.
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PMID:OPD4-positive T-cell lymphoma of the liver in systemic lupus erythematosus. 178 43

The clinicopathologic features of 23 patients with hematophagic histiocytosis (HH) are described. All of them exhibited increased histiocytes associated with hemophagocytosis in the marrow. The patients usually presented with fever, hepatosplenomegaly, lymphadenopathy, and cytopenia. The underlying illnesses were heterogeneous, including non-Hodgkin's lymphoma in 17, systemic lupus erythematosus in one, diabetes mellitus in one, acute myelomonocytic leukemia in one, myelodysplastic syndrome in one, and unknown cause in two. Among 17 non-Hodgkin's lymphoma, 14 were peripheral T-cell lymphoma, two were B-cell lymphoma, and one was an undefined phenotype. Among 14 patients with peripheral T-cell lymphoma, six of the patients had nasal T-cell lymphoma. Five of these 14 patients initially diagnosed as malignant histiocytosis turned out to be T-lineage lymphoma after immunophenotypic studies. Active infections, most of viral origin, were documented in eight patients, including Epstein-Barr virus in three, cytomegalovirus in three, herpes simplex virus in three, Pseudomonas aeruginosa in one, Bacteroides vulgatus in one, and mycoplasma in one. Some of them had mixed virus and bacteria infection. Sixteen (70%) of our patients died of their acute illness within 10 weeks of the diagnosis of HH. In the past, the clinical and histologic differentiation between hematophagic histiocytosis and true histiocytic neoplasm (histiocytic medullary reticulosis/malignant histiocytosis) has proved difficult, but now these can be distinguished with immunohistologic, immunogenetic, and cytogenetic studies, especially in the cases of peripheral T-cell lymphoma with hemophagocytic syndrome.
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PMID:Hematophagic histiocytosis: a clinicopathologic analysis of 23 cases with special reference to the association with peripheral T-cell lymphoma. 792 83

Anti-endothelial cell antibodies in systemic lupus erythematosus were further characterized by controlled immunoblotting studies with EN4 defined membrane and cytosol preparations of human umbilical vein endothelial cells. Antibodies to endothelial cell membranes, some of which reacted with the membranes of both dermal fibroblasts and T-cell lymphoma HUT78, were detected in 26/33 patients (78%), but in only 4/34 normal controls (P < 0.001) and 3/11 patients with a recent myocardial infarction. Although the antibody response was very heterogeneous against epitopes ranging from 17 to 205 kDa, there was a tendency to detect particular membrane epitopes at 31-33 kDa (15 cases), 72-78 kDa (eight cases), 66-68 kDa (seven cases) and 17-19 kDa (five cases). No correlations between antibodies to particular epitopes and disease manifestations were observed nor was a relationship to disease activity detected in a retrospective analysis. However, the possibility that anti-endothelial cell antibodies may be pathogenically important was supported by prospective serial studies in two cases with nephritis who showed diminution and disappearance of anti-endothelial cell antibodies as their active disease was treated into remission.
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PMID:Further characterization of anti-endothelial cell antibodies in systemic lupus erythematosus by controlled immunoblotting. 901 49

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.
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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.
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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.
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PMID:Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients. 1290 41

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.
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PMID:[Lupus panniculitis]. 1579 39

We conducted clinicopathologic and immunohistochemical analyses of the prevalence of Epstein-Barr virus (EBV) among 24 patients with malignant lymphoma complicating systemic rheumatic diseases. (SRD) These 24 patients included 17 with rheumatoid arthritis (RA), 3 with systemic lupus erythematosus (SLE), 2 with systemic sclerosis (SS), and 2 with dermatomyositis (DM). There were 2 men and 22 women ranging in age from 30 to 86 years (mean: 64 years). The interval between the onset of rheumatic disease and that of malignant lymphomas ranged from 3 months to 35 years (mean: 142 months). The use of immunosuppressive drugs before the onset of malignant lymphoma was recorded in 15 patients. Among them, 5 patients received methotrexate (MTX) therapy. Malignant lymphomas were found at extranodal sites in 9 patients, and the disease was in the advanced stage in 17 patients. Histologic and immunohistochemical studies demonstrated that 18 cases (75%) were B-cell lymphoma (RA=12, SLE=2, SS=2, DM=2), 3 (12.5%) were peripheral T-cell lymphoma (RA=3), and 3 (12.5%) were classical Hodgkin lymphoma (RA=2, SLE=1). As in previous reports, there was an increased frequency of diffuse large B-cell lymphoma (50%) in the present series. Moreover, a majority of the diffuse large B-cell lymphomas exhibited activated B-cell phenotype. EBV-encoded small RNAs (Epstein-Barr early region [EBER]-) and/or LMP-1+tumor cells were identified in only 3 cases of classical Hodgkin lymphomas. Our findings suggested EBV-associated lymphoma comprised only a small fraction of all non-Hodgkin's lymphomas in the general SRD patient population.
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PMID:Malignant lymphoma in patients with systemic rheumatic disease (rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, and dermatomyositis): a clinicopathologic study of 24 Japanese cases. 1650 34

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.
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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.
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PMID:Lupus erythematosus panniculitis. 1879 77


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