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)

A large variety of vasculopathic syndromes are uncommonly associated with malignancies. Vasculitis is usually manifested by skin lesions and is generally associated with hematologic malignancies rather than solid tumors. Evidence of autoantibodies, immune complexes, and complement consumption is typically absent. Myelodysplastic syndromes can be confidently linked to vasculitis on the basis of recent literature. The temporal relationship of malignancy to vasculitis development is variable except that vasculitis generally follows the discovery of hairy cell leukemia and splenectomy. Vasculitis may occasionally be a complication of chemotherapy, radiation therapy, and bone marrow transplantation. Occasionally, malignant disorders may mimic vasculitic syndromes. The etiopathogenesis of vasculitis in patients with malignant disorders is unknown. The recent literature on vasculitis and malignancy addresses predominantly case reports and small patient cohorts and identifies clinical characteristics rather than pathogenic mechanisms.
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PMID:Vasculitis associated with malignancy. 154 62

Pseudomonas pneumonia is one of the fatal opportunistic infections. We examined Pseudomonas aeruginosa in the autopsied lung tissues by the immunostaining (Biotin-Streptoavidin peroxidase complex method). Out of 185 autopsied cases, fifty-three cases had bacterial pneumonia. We performed the immunostaining to these 53 cases, of which 14 cases (26.4%) were diagnosed as pseudomonas pneumonia. All of the 14 cases had severe underlying diseases which were hematologic malignancies in six, solid malignancies in five and so on. Of ten of them pseudomonas pneumonia was considered to be the direct cause of death. P. aeruginosa was observed in alveolar space in all cases, and in bronchial and bronchiolar lumen in many cases. P. aeruginosa was also detected in alveolar and vascular walls. Histopathologically necrotizing vasculitis and alveolar destruction were characteristic, and intra-alveolar hemorrhage, fibrinous exudate, abscess formation, coagulation necrosis and thrombosis were observed in most cases. Necrotizing vasculitis and intra-alveolar hemorrhage were more severe in the cases of leukopenic patients.
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PMID:[A histopathological study on pseudomonas pneumonia diagnosed by immunostaining with monoclonal antibody]. 250 18

Pyoderma gangrenosum and Sweet's syndrome are classified as neutrophilic dermatoses as they exhibit intense dermal inflammatory infiltrates composed of neutrophils with little evidence of a primary vasculitis. They share several characteristics and respond to immunosuppressives. Aetiology is felt to represent a manifestation of altered immunologic reactivity. Patients with both conditions concurrently have been described. Diagnosis is based on clinical and histopathological findings. However, clinically the typical forms of the two conditions are quite distinct: pyoderma showing cutaneous ulceration with a purple undermined border and Sweet's syndrome having tender, erythematous, nonulcerated plaques and nodules. Approximately 50% of cases of pyoderma are associated with a specific systemic disorder. These include inflammatory bowel disease, rheumatoid arthritis, non-Hodgkin's lymphoma and myeloproliferative disorders. Many associations with Sweet's syndrome have been described, including acute myeloid leukaemia, myeloma and adenocarcinomas, and haematological malignancy. There is overlap between the two conditions with lesions categorised as Sweet's syndrome being clinically more characteristic of atypical pyoderma and vice versa. We believe that pyoderma and Sweet's syndrome represent a continuum of spectrum of disease. The reason for the clinical differences between the conditions is unclear and merits further investigation but may be explained by varying levels of intensity and extent of the inflammatory process. This review will describe the pathogenesis, clinical features, diagnosis, associations and treatment of the two conditions.
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PMID:Neutrophilic dermatoses: pyoderma gangrenosum and Sweet's syndrome. 912 99

Mucocutaneous paraneoplastic syndromes represent a group of dermatoses of variable morphology, pathology, and etiology that can occur as idiopathic conditions or in association with a visceral malignancy. These conditions can be categorized with respect to their predominant pathologic change: dermal "depositions," neutrophilic dermatoses, papulosquamous disorders, proliferative reactions, reactive erythemas, vacuolar degeneration of the basal layer, vasculitis, and vesiculobullous disorders. Some of these dermatoses occur more frequently in patients with hematologic malignancies whereas others are more prevalent in patients with solid tumors. The major clinical characteristics and commonly associated malignancies in patients with mucocutaneous paraneoplastic syndromes are reviewed. Suggested workups to evaluate for cancer in patients with these dermatoses are summarized. The appearance of a mucocutaneous paraneoplastic syndrome can either precede, occur concurrently with, or follow the detection of an associated neoplastic process. Therefore, the dermatosis can be the presenting feature of a previously unsuspected neoplasm or the earliest sign of recurrent cancer in an oncology patient. When the possibility of a mucocutaneous paraneoplastic syndrome is entertained, the diagnosis of the dermatosis should be confirmed either based on the clinical morphology of the lesions, the pathologic changes observed after lesional biopsy, or both. Once the diagnosis of a malignancy-associated dermatosis has been established, either an appropriate evaluation for an asymptomatic neoplasm in a cancer-free individual or an investigation for recurrence of malignancy in an oncology patient can be initiated.
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PMID:Mucocutaneous paraneoplastic syndromes. 920 89

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the brain caused by the JC virus. It occurs in association with immunodepression due, for instance, to a hematological malignancy, HIV infection, or immunosuppressive therapy for an organ transplant or systemic disease. We describe the fourth reported case of PML in a patient receiving immunosuppressants for Wegener's granulomatosis. A 71-year-old woman receiving azathioprine and glucocorticoid therapy experienced onset of right-sided hemiplegia within a few days, became comatose, and died within a few days. MRI of the brain showed a subcortical lesion in the left parietal lobe generating low signal on T1 images and high signal on T2 images. The initial diagnosis was cerebral vasculitis. However, the postmortem examination showed PML. The diagnosis of PML rests on JC virus detection in the cerebrospinal fluid by PCR assay and on demonstration in a brain biopsy of the typical histological pattern with presence of the JC virus within the demyelinated lesions. No specific or effective treatments are available. Immunosuppressant drugs should be discontinued if possible.
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PMID:JC virus leukoencephalopathy complicating Wegener's granulomatosis. 1456 68

Lymphocytic cutaneous vasculitis associated with a haematological malignancy has rarely been reported. Here, we describe a 61 year-old woman with chronic lymphocytic leukemia (CLL) who presented with cutaneous lesions on both hands. These lesions improved after all combination chemotherapy courses and recurred before each course. Repetitive skin biopsies revealed lymphocytic vasculitis. After 7 courses of chemotherapy, she had a complete remission. Skin lesions disappeared and did not recur. The cyclic pattern of lymphocytic vasculitis and its relation with CLL disease activity are interesting clinical features in this case.
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PMID:Cyclic lymphocytic vasculitis associated with chronic lymphocytic leukemia. 1516 Sep 61

Pyoderma gangrenosum (PG) is an uncommon cutaneous disease of unknown etiology. In 50 percent of affected patients, PG is associated with systemic disease including inflammatory bowel disease, arthritis, and hematologic malignancies.(1) Diagnosis of PG is based on clinical presentation, histopathology and on the exclusion of other diseases that can produce clinically similar lesions, e.g. infection, vasculitis, malignancy, collagen vascular diseases, diabetes, and trauma. Four variants of PG have been described: ulcerative, pustular, bullous, and vegetative.(2) We report a woman with renal failure who developed PG in the absence of any obvious triggering trauma in a distinctive unilateral crop just distal to an arteriovenous dialysis shunt.
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PMID:Pyoderma gangrenosum occurring near an arteriovenous dialysis shunt. 1686 25

Langerhans' cell histiocytosis (LCH) is a rare condition of children and young adults in which Langerhans' cells proliferate. The clinical spectrum ranges from solitary or few focal lesions to multisystem involvement mimicking vasculitis or hematological malignancy. Focal bone lesions, known as eosinophilic granulomas, are the most common manifestations. Eosinophilic granuloma usually presents with a variable combination of pain, swelling, fracture, and fever. Facial bone involvement may manifest as an ear discharge, hearing loss, or exophthalmos. Nerve root pain is rarely reported, even in patients with lesions in the axial skeleton. We report four cases of nerve root pain caused by LCH. Two male patients aged 25 and 34 years, respectively, presented with truncated femoral neuralgia related to acetabular granulomas. A 25-year-old woman with involvement of the L5 vertebral body and a 41-year-old man with a sacral lesion presented with sciatica.
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PMID:Radiculopathy as a manifestation of Langerhans' cell histiocytosis. 1734 85

To detect and describe the incidence of musculoskeletal manifestations in different malignant diseases as well as their relation to the treatment received whether by chemotherapy or radiation therapy. Sixty patients with different malignant diseases were included in this study, 45 with solid tumors and 15 patients with hematological malignancy. The mean age was 46.55 +/- 11.04 years and the mean disease duration was 2 +/- 0.75 years. The patients were fully examined for any rheumatologic involvement, laboratory investigations were performed as well as dual energy X-ray absorptiometry study for bone densitometry. Treatment strategies were assessed including the chemotherapeutics, radiation therapy, and/or surgery. Myalgias and arthralgias were the most frequent followed by flexor tenosynovitis, frozen shoulder, and fibromyalgia syndrome. Hypertrophic osteoarthropathy was seen in five patients, cutaneous vasculitis in two patients as well as arthritis. Osteonecrosis was present in one of the lunate carpal bones of a patient with non-Hodgkin's lymphoma (1.67%) and receiving high dose steroids. Rheumatoid factor was positive in four patients, three of which had hepatitis C virus positivity and cryoglobulins. Anti-neutrophil cytoplasmic antibody was negative in all the studied patients. The bone mineral density was significantly reduced in the patients with malignancy compared to the control. Mild to moderate osteoporosis was present, being more evident in the spine and forearm. The bone loss was higher in those with solid tumors and even more obvious in those receiving aromatase inhibitors. Musculoskeletal manifestations occurring during malignancies and following the treatment represent a significant percentage of symptoms and signs which may raise a clue to differential diagnosis.
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PMID:Musculoskeletal manifestations in patients with malignant disease. 1989 74

Acquired eosinophilia is operationally categorized into secondary, clonal, and idiopathic types. Causes of secondary eosinophilia include parasite infections, allergic or vasculitis conditions, drugs, and lymphoma. Clonal eosinophilia is distinguished from idiopathic eosinophilia by the presence of histologic, cytogenetic, or molecular evidence of an underlying myeloid malignancy. The World Health Organization classification system for hematologic malignancies recognizes 2 distinct subcategories of clonal eosinophilia: chronic eosinophilic leukemia, not otherwise specified and myeloid/lymphoid neoplasms with eosinophilia and mutations involving platelet-derived growth factor receptor alpha/beta or fibroblast growth factor receptor 1. Clonal eosinophilia might also accompany other World Health Organization-defined myeloid malignancies, including chronic myelogenous leukemia, myelodysplastic syndromes, chronic myelomonocytic leukemia, and systemic mastocytosis. Hypereosinophilic syndrome, a subcategory of idiopathic eosinophilia, is defined by the presence of a peripheral blood eosinophil count of 1.5 x 10(9)/L or greater for at least 6 months (a shorter duration is acceptable in the presence of symptoms that require eosinophil-lowering therapy), exclusion of both secondary and clonal eosinophilia, evidence of organ involvement, and absence of phenotypically abnormal and/or clonal T lymphocytes. The presence of the latter defines lymphocytic variant hyper eosinophilia, which is best classified under secondary eosinophilia. In the current review, we provide a simplified algorithm for distinguishing the various causes of clonal and idiopathic eosinophilia and discuss current therapy, including new drugs (imatinib mesylate, alemtuzumab, and mepolizumab).
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PMID:Hypereosinophilic syndrome and clonal eosinophilia: point-of-care diagnostic algorithm and treatment update. 2005 13


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