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Multiorgan Involvement (multiorgan + involvement)
Selected AbstractsUnsuspected systemic amyloidosis diagnosed by fine-needle aspiration of the salivary gland: Case reportDIAGNOSTIC CYTOPATHOLOGY, Issue 1 2004Ph.D., Tamar Giorgadze M.D. Abstract Amyloidosis of the head and neck region may represent a local amyloidoma or a manifestation of systemic disease. Involvement of major salivary glands by either primary or secondary forms of amyloidosis is very rare. We describe a case of systemic amyloidosis that initially presented as submandibular gland mass and was diagnosed by fine-needle aspiration (FNA). A 69-year-old male presented with submandibular mass. His past medical history was significant for left forearm melanoma that was excised 6 years ago and tricuspid valve endocarditis after valvular replacement 3 months prior to FNA of the submandibular gland. The patient had no symptoms or clinical and laboratory data suggestive of amyloidosis. FNA specimen showed salivary gland tissue and abundant amorphous material, which stained positive for amyloid with Congo red stain and showed typical birefringence when examined by polarized microscopy. Further workup of the patient revealed generalized amyloidosis with multiorgan involvement by the disease. This case demonstrates that FNA can be a useful technique in the diagnosis of unsuspected amyloidosis. Diagn. Cytopathol. 2004;31:57,59. © 2004 Wiley-Liss, Inc. [source] An Analysis of Possible Mechanisms of Unexpected Death Occurring in Hydatid Disease (Echinococcosis)JOURNAL OF FORENSIC SCIENCES, Issue 4 2009Roger W. Byard M.B.B.S. Abstract:, Most cases of hydatid disease in human populations are due to Echinococcus granulosus. The hydatid life cycle involves passage between definitive hosts such as dogs and intermediate hosts such as sheep. Humans become accidental intermediate hosts following ingestion of food or water contaminated with eggs or by contact with infected dogs. Although hydatid disease may remain asymptomatic, occasional cases of sudden and unexpected death present to autopsy. Causes of rapid clinical decline involve a wide range of mechanisms including anaphylaxis (with or without cyst rupture), cardiac outflow obstruction or conduction tract disturbance, pulmonary and cerebral embolism, pericarditis, cardiac tamponade, myocardial ischemia, pulmonary hypertension, peritonitis, hollow organ perforation, intracerebral mass effect, obstructive hydrocephalus, seizures, cerebral ischemia/infarction, and pregnancy complications. The autopsy assessment of cases therefore requires careful examination of all organ systems for characteristic cystic lesions, as multiorgan involvement is common, with integration of findings so that possible mechanisms of death can be determined. Measurement of serum tryptase and specific IgE levels should be undertaken for possible anaphylaxis. [source] Clinicopathlogical features and prognosis of drug rash with eosinophilia and systemic symptoms: a study of 30 cases in TaiwanJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 9 2008C-C Chiou Abstract Background, Drug rash with eosinophilia and systemic symptoms (DRESS), a group of non-blistering severe cutaneous adverse drug reactions (SCADRs), is characterized by skin rash and multiorgan involvement. Details of this reaction have not been reported in the literature so far. Aim, We investigate clinical and pathological features and prognosis of DRESS and hope this study will provide data concerning this disorder in Taiwan. Methods, From January 2001 to June 2006, a total of 30 patients, diagnosed with DRESS, were enrolled and evaluated for demographic characteristics, pathological findings, complications and outcome. Results, Patient ages ranged from 13 to 78, with an equal sex ratio. The most common offending drug was allopurinol followed by carbamazepine. Pathologic changes observed were lichenoid dermatitis, erythema multiforme, pseudolymphoma and vasculitis. Impairment of liver and renal functions and blood dyscrasia were frequent complications. Active infection or reactivation of HHV-6 was observed in 7 of 11 patients studied serologically. Two patients developed type 1 diabetes mellitus. The mortality rate was 10% (3 of 30). Conclusions, DRESS is a heterogeneous group of life-threatening conditions. The leading drug in DRESS in Taiwan is allopurinol. High eosinophil count and multiple underlying diseases are poor prognostic factors in patients with DRESS. [source] Sera from anti,Jo-1,positive patients with polymyositis and interstitial lung disease induce expression of intercellular adhesion molecule 1 in human lung endothelial cellsARTHRITIS & RHEUMATISM, Issue 8 2009Sevim Barbasso Helmers Objective To investigate whether sera or purified IgG from patients with polymyositis (PM) and patients with dermatomyositis (DM), with or without interstitial lung disease (ILD), can activate endothelial cells (ECs). Methods Patients' sera were selected based on the presence or absence of anti,Jo-1, anti-SSA, or anti,U1 small nuclear RNP autoantibodies. The presence of autoantibodies was determined by line blot assays. Cultured human microvascular ECs derived from lung tissue (HMVEC-L) were incubated with sera or purified IgG from 22 patients with PM, 7 patients with DM, and 10 healthy individuals as controls. Assessment of intercellular adhesion molecule 1 (ICAM-1) expression was conducted by immunofluorescence (n = 22) and by cell-based enzyme-linked immunosorbent assay (ELISA) (n = 20). Serum levels of soluble ICAM-1 (sICAM-1) were determined by ELISA. Results Sera from PM patients with ILD who were positive for anti,Jo-1 autoantibodies had a significantly stronger effect on the expression of ICAM-1 by HMVEC-L in comparison with sera from healthy controls and patients with other autoantibodies. Purified IgG did not induce ICAM-1 expression. Higher serum levels of sICAM-1 were found in patients with myositis compared with healthy controls. Conclusion EC activation with ICAM-1 expression could contribute to the multiorgan involvement, including the development of myositis and ILD, in patients carrying anti,Jo-1 autoantibodies. The EC-activating factors are not the autoantibodies themselves, but might be systemic factors associated with these autoantibodies. [source] Langerhans cell histiocytosis associated with breast carcinoma successfully treated with topical imiquimodCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 8 2009D. O'Kane Summary Langerhans cell histiocytosis (LCH) encompasses a group of disorders characterized by the proliferation and infiltration of Langerhans cells within internal organs and/or skin. There is often multiorgan involvement; isolated cutaneous LCH is less common.1 The aetiology of cutaneous LCH remains uncertain, and debate remains as to whether LCH represents a neoplastic condition or is simply reactive. We report a 53-year-old woman who developed isolated cutaneous LCH 15 months after being diagnosed with infiltrating ductal carcinoma of the left breast. The LCH was treated with topical imiquimod, resulting in clinical and histological resolution. Our case highlights the rare association between cutaneous LCH and breast carcinoma, and the clinical and histological response that can be achieved with topical imiquimod. After a diagnosis of LCH, patients require long-term follow-up, due to the risk of recurrence and/or development of a subsequent malignancy. [source] |