Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In summary, Wegener's granulomatosis is a systemic illness that is characterized pathologically by necrotizing granulomatosis and vasculitis. Lethal midline granuloma is not a pathological term. Lethal midline granuloma is a clinical term, and in reality, lethal midline granuloma has been proven to be polymorphic reticulosis and not Wegener's granulomatosis. Polymorphic reticulosis is now understood to be a T-cell lymphoma. Further study of these malignant lymphomas has shown that these T-cell lymphoproliferative disorders may be causally associated with Epstein-Barr virus. Although there is strong association between autoantibodies against cytoplasmic constituents of neurophils and monocytes in patients with active Wegener's granulomatosis, the exact pathogenic mechanism in Wegener's granulomatosis and the etiology is still unknown. IgG-C-ANAC (C-ANAC stands for Classical/Cytoplasmic Anti-Neutrophil Cytoplasm Antibodies) increases with increasing disease activity from undetectable levels to up to 95% of active Wegener's granulomatosis patients. Rhinologic symptoms in Wegener's granulomatosis include progressive nasal obstruction, bloody rhinorrhea with crusting, and vague pain and tenderness of the nasal dorsum. Usually these patients have mucosal ulcerations of the nose with or without a septal perforation. Other areas of the head and neck can be involved, and they include orbital, otologic, oral, and subglottic involvement. Hallmarks of malignant lymphoma (polymorphic reticulosis) when it involves the upper airway include rapid localized destruction of the nose, orbit, paranasal sinuses, and hard palate. Treatment for Wegener's granulomatosis includes antimicrobial agents in addition to a regimen of cyclophosphamide and glucocorticoids. The treatment for malignant lymphoma (polymorphic reticulosis) is primarily radiation, especially when confined to one site.
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PMID:Fireside conference 19. Wegener's granulomatosis and lethal midline granuloma. 138 98

Most neurosurgeons and neurologists still consider lymphomatoid granulomatosis (LG) as a type of central nervous system (CNS) vasculitis. However, new insights, primarily from the hematologic literature, have cast doubt on the benign character of this disorder. In this article we (1) report a case of an 18-year-old woman with diffuse CNS disease and no mass lesion who developed multiple small cortical infarcts and dementia secondary to multifocal angiocentric, angiodestructive lymphoma; we (2) review other cases of LG with predominant CNS involvement; we (3) summarize the current understanding of LG, which is now considered to be a premalignant or overt angiocentric, angiodestructive T-cell lymphoma rather than a non-neoplastic vasculitis: the importance to neurologic surgeons and neurologists is that while pulmonary involvement in LG is generally the most prominent finding, patients may present with early or dramatic CNS disease; and we (4) note that although dementia is uncommon in young adults, this report adds yet another rare condition to the long differential list of dementia in this age group.
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PMID:Central nervous system angiocentric, angiodestructive T-cell lymphoma (lymphomatoid granulomatosis). 154 76

Three patients with extranodal peripheral T-cell lymphoma and a distinctive clinical presentation are described. They had acute onset of fever, weight loss, progressive liver failure, bleeding diathesis, pancytopenia, and myelodysplastic changes in the bone marrow. Each patient had one or more paraneoplastic complications: severe rhabdomyolysis with myoglobinuria and secondary renal failure, cutaneous vasculitis, gluten-sensitive enteropathy, polyserositis, and increased macrophages with hemophagocytic activity. They did not have peripheral lymphadenopathy. The complex clinical presentations simulated collagen vascular disorders, systemic infections, or severe liver disease rather than a malignant lymphoma. Routine histologic studies revealed a small population of lymphoma cells in the bone marrow, spleen, and liver. Immunophenotyping studies demonstrated their T-cell phenotype, and cytogenetic analysis showed the clonality in Patients 1 and 2; clonal T-cell receptor gene rearrangement was found in Patients 2 and 3. These studies should be considered in the evaluation of patients with constitutional symptoms, liver failure, coagulopathy, and pancytopenia even in the absence of peripheral lymphadenopathy.
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PMID:Unusual presentation of extranodal peripheral T-cell lymphomas with multiple paraneoplastic features. 164 87

In two patients a peripheral neuropathy was the presenting symptom of a noncutaneous peripheral T-cell lymphoma. In the first patient, the neuropathy had a relapsing and remitting course, the symptoms improved under corticosteroid therapy. The second patient suffered from a relentless neuropathy. In both cases the lymphoma infiltrated the peroneal nerve with an angiocentric and perivascular pattern resembling that observed in central nervous system lymphomas. The characterization of T-cell subsets in the lymph node showed cells with the helper/inducer and suppressor/cytotoxic phenotype in the first case and a predominance of cells with the helper/inducer phenotype in the second case. In the nerve, lymphocytes beard the helper/inducer phenotype antigen. A typical paraneoplastic vasculitis of nerve showed clearly different immunologic features.
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PMID:T-cell lymphoma revealed by a peripheral neuropathy. A report of two cases with an immunohistologic study on lymph node and nerve biopsies. 302 11

The association between cutaneous vasculitis and lymphoproliferative disease has been increasingly recognized. We report a female patient who presented with cutaneous vasculitis which was due to a small cell pleomorphic T-cell lymphoma. In spite of aggressive therapy, she died two years after onset of disease. The clinical picture of vasculitis associated with lympho-proliferative disease is addressed with particular emphasis on symptoms and signs suggesting malignancy rather than vasculitis appearing in concert with well-defined connective tissue disorders.
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PMID:Small cell pleomorphic T-cell lymphoma presenting with cutaneous vasculitis. 769 68

Human T-cell lymphotropic virus type I (HTLV-I) is known to cause adult T-cell leukemia/T-cell lymphoma and tropical spastic paraparesis/HTLV-I-associated myelopathy. Recent seroepidemiologic, clinical, and virologic studies indicate that the virus is also related to a certain type of uveitis, which has been classified as uveitis without defined etiologies or idiopathic uveitis. According to the seroepidemiologic survey, the seroprevalence of HTLV-I in patients with idiopathic uveitis was significantly higher than that of two control groups, that is, patients with uveitis with defined etiologies and patients with nonuveitic ocular diseases. Clinically, the uveitis seen in HTLV-I carriers is characterized by moderate to severe cellular infiltration in the eye and by moderate retinal vasculitis, and the intraocular inflammation responds well to corticosteroid therapy. Interestingly, 25% of female patients with the disease had a previous history of Graves disease with hyperthyroidisms. The following virologic, molecular biologic findings suggest that cytokines produced by HTLV-I-infected T cells in the eye play the central role in the pathogenic mechanisms of the uveitis: (a) the virus load in the peripheral blood monocytes analyzed by the quantitative polymerase chain reaction methods was significantly greater in patients with the uveitis than in asymptomatic carriers, (b) the proviral DNA of HTLV-I and the gene expression of the virus at the mRNA level was detected in the infiltrating cells from the eyes of the patients, (c) the virus particles were detected by electron-microscopic examination in the T-cell clones established from the intraocular fluid of the patients, and (d) the HTLV-I-infected T cells produced a variety of cytokines without any stimuli, such as interleukin (IL)-1 alpha, IL-2, IL-3, IL-6, IL-8, IL-10, tumor necrosis factor alpha, interferon-gamma, and granulocyte-macrophage colony-stimulating factor. Based on the seroepidemiologic, clinical, and virologic data, the uveitis seen in HTLV-I carriers is considered to be a distinct clinical entity related to HTLV-I infection, and the disease is designated as HTLV-I uveitis.
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PMID:HTLV-I uveitis. 879 4

Apoptosis was suspected to play a significant role in spontaneous regression of skin lesions in four cases of peripheral T-cell lymphoma arising in the skin or subcutis. All of the patients had skin tumors with ulcer formation, but no metastasis to the lymph nodes or viscera. Biopsy showed a sea of CD3-positive lymphoma cells involving the dermis to the subcutis with scattered foci of coagulative necrosis. In the zone of incipient necrosis surrounding the core of coagulative necrosis, frequent apoptotic bodies were identified by electron microscopy (EM), and DNA strand breaks were detected in 34% to 51% (mean, 42%) of lymphoma cells by the TdT-mediated deoxyuridine triphosphate (dUTP)-biotin nick end-labeling (TUNEL). As for the pathogenesis of coagulative necrosis, ischemic necrosis attributable to lymphocytic vasculitis appeared unlikely, because necrotic foci were devoid of neutrophils and had no relation to vascular distribution. Primary cataclysmic apoptosis appeared more likely, but its pathogenetic role could not be established, because EM and TUNEL could not be applied to the core of the debris-ridden coagulative necrosis. Although these cases had been classified as pseudolymphomas because they were histologically malignant but clinically benign, they were in fact true lymphomas characterized by extensive coagulative necrosis with a high rate of apoptosis demonstrable in the zone of incipient necrosis.
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PMID:Role of apoptosis in spontaneous regression of peripheral T-cell lymphoma arising in the skin or subcutis. 910 48

Biopsy from the edge of a septal perforation is performed to diagnose potentially significant aetiological conditions, including malignant pathology and Wegener's granulomatosis. However, it is common for the histological result to be undiagnostic and unhelpful in planning an individual patient's management. To clarify the role of septal perforation biopsy, we reviewed all biopsies performed on our patients over a 13-year period. Of the 65 biopsies performed, in 63 patients, none had a histological result which changed the clinical diagnosis made before biopsy. Forty-three patients had clinically benign perforations biopsied, and no histology other than chronic inflammation was obtained. In 16 patients with a clinical vasculitis, none had this diagnosis confirmed histologically. One positive biopsy was obtained from two patients with clinically malignant perforations yet both were treated for T-cell lymphoma. The subsequent management of all patients was based on their clinical diagnosis rather than their biopsy results. We suggest only clinically malignant perforations are worthwhile biopsying.
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PMID:The clinical value of septal perforation biopsy. 1081 12

Superantigens have been suggested to play an important role in the pathogenesis of several inflammatory skin diseases as well as systemic diseases such as atopic dermatitis, psoriasis, vasculitis, T-cell lymphoma and autoimmune diseases. Infections often precede the onset and relapse of these diseases, and antibiotic treatment with or without additional glucocorticosteroids and immunoglobulins is occasionally successful. Superantigens are microbial proteins that are able to stimulate up to 20% of the naive T-cell population in a non-specific way. They are produced by gram-positive and -negative bacteria as well as by viruses, parasites and yeasts. The importance of the pathogenic role of superantigens is determined by the potency to induce inflammation by extensive cytokine release after T-cell stimulation and/or T-cell-mediated cytotoxicity and, thereby, tissue damage. Furthermore, superantigens may be able to induce autoimmune processes by stimulation of autoreactive T-cells as well as autoantibody production by stimulation of B-cells. Among the diseases associated with superantigens, atopic dermatitis, guttate and chronic plaque psoriasis, as well as Kawasaki disease, are by far the best-characterized. Nevertheless, conflicting results have been obtained and formal proof of a pathogenic role of superantigens in these diseases is still lacking. The aim of this review is to summarize the data on superantigens in terms of their distribution in microorganisms, their interactions with the adaptive immune system and their contribution to skin pathology.
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PMID:Superantigens and their association with dermatological inflammatory diseases: facts and hypotheses. 1120 Aug 27

We describe two Korean adult patients who had necrotizing papulovesicles mainly on their faces. Skin biopsy specimens showed perivascular and periadnexal infiltrate of atypical lymphoid cells with vasculitis in the dermis and subcutaneous tissue. In situ hybridization demonstrated a latent infection of Epstein-Barr virus in the majority of lymphoid cells in the dermis. These patients were diagnosed as having T-cell lymphoma. Interestingly, large granular lymphocytosis was found in the peripheral blood of Case 2.
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PMID:Epstein-Barr virus-associated peripheral T-cell lymphoma in adults with hydroa vacciniforme-like lesions. 1142 65


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