Idiopathic pulmonary haemosiderosis. Epidemiology, pathogenic aspects and diagnosis.
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Idiopathic pulmonary haemosiderosis (IPH) is a rare clinical entity characterized by recurrent episodes of diffuse alveolar haemorrhage, often presenting with haemoptysis. Many patients have iron deficiency anaemia due to deposition of haemosiderin iron in the alveoli, and eventually develop moderate pulmonary fibrosis. Typically, intensive search for an aetiology ends up negative. There is no evidence of pulmonary vasculitis or capillaritis. The aetiology is obscure, but may be an immunological or toxic mechanism causing a defect in the basement membrane of the pulmonary capillary. IPH affects both children and adults. During an acute episode, a chest X-ray demonstrates bilateral, alveolar infiltrates. Sputum examination discloses haemosiderin-laden alveolar macrophages. Diagnosis is established by lung biopsy (fiber-optic or thoracoscopic), showing large numbers of haemosiderin-laden macrophages in the alveoli and without evidence of capillaritis or deposition of immunoglobulins. Corticosteroids and/or immunosuppressive drugs may be effective during an acute bleeding episode, and may in some patients improve symptoms and prognosis on the long-term, but the response to treatment displays great interindividual variation. (+info)
Overview of investigations into pulmonary hemorrhage among infants in Cleveland, Ohio.
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Idiopathic pulmonary hemorrhage was diagnosed in 37 infants in the Cleveland, Ohio, area between 1993 and 1998. This rare disorder has been related to 12 deaths, including 7 originally thought to be sudden infant death syndrome. Thirty of the infants were African American, all of whom lived in a limited geographic area of eastern metropolitan Cleveland, an area of older housing stock. An investigation led by the Centers for Disease Control and Prevention has found an association with household exposure to a toxigenic mold, Stachybotrys chartarum, and other fungi. The rapidly growing lungs of young infants appear to be especially vulnerable to the toxins made by toxigenic molds. Environmental tobacco smoke was frequently present in the infants' homes and may be a trigger precipitating the acute bleeding. Stachybotrys, although not thought to be a common mold, is known to have a wide geographic distribution. An additional 101 cases of acute, idiopathic pulmonary hemorrhage have been reported in infants in the United States over the past 5 years. In this overview, the investigations are summarized, the clinical profile is described, the toxicity of S. chartarum is discussed, and pathophysiologic concepts are presented. (+info)
Anthracycline cardiotoxicity in a black rhinoceros (Diceros bicornis): evidence for impaired antioxidant capacity compounded by iron overload.
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Two weeks before dying of congestive heart failure, a juvenile black rhinoceros (Diceros bicornis minor) received a single low dose of doxorubicin as part of combination chemotherapy for acute lymphoblastic leukemia. Diffuse hemosiderosis was present at necropsy in a pattern indicative of dietary iron overload, but unique iron-positive degenerative lesions were found in isolated myocardiocytes. Serum analyses revealed hyperferremia, 87% transferrin saturation, and 5- to 10-fold elevations in ferritin concentration, reflecting markedly increased tissue iron stores. Since both toxic and therapeutic effects of anthracyclines are mediated by formation of reactive free radicals via iron-catalyzed reactions, these observations suggest that iron overload may have enhanced myocardial susceptibility to cardiotoxic effects of doxorubicin. Impairments in other myocardial antioxidant defenses, such as deficiencies in catalase and glutathione S-transferase that are known to exist in rhinoceros erythrocytes, may have been underlying factors contributing to an inherent sensitivity of rhinoceros tissues to oxidant-induced injury. (+info)
Quantification of siderophore and hemolysin from Stachybotrys chartarum strains, including a strain isolated from the lung of a child with pulmonary hemorrhage and hemosiderosis.
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A strain of Stachybotrys chartarum was recently isolated from the lung of a pulmonary hemorrhage and hemosiderosis (PH) patient in Texas (designated the Houston strain). This is the first time that S. chartarum has been isolated from the lung of a PH patient. In this study, the Houston strain and 10 strains of S. chartarum isolated from case (n = 5) or control (n = 5) homes in Cleveland were analyzed for hemolytic activity, siderophore production, and relatedness as measured by random amplified polymorphic DNA analysis. (+info)
Juvenile hemochromatosis associated with B-thalassemia treated by phlebotomy and recombinant human erythropoietin.
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Juvenile hemochromatosis is a rare genetic disorder that causes iron overload. Clinical complications, which include liver cirrhosis, heart failure, hypogonadotropic hypogonadism and diabetes, appear earlier and are more severe than in HFE-related hemochromatosis. This disorder, therefore, requires an aggressive therapeutic approach to achieve iron depletion. We report here the case of a young Italian female with juvenile hemochromatosis who was unable to tolerate frequent phlebotomy because of coexistent ss-thalassemia trait. The patient was successfully iron-depleted by combining phlebotomy with recombinant human erythropoietin. (+info)
Diagnostic accuracy of abdominal ultrasonography compared to magnetic resonance imaging in siderosis of the spleen.
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A prospective study to compare the diagnostic performance of ultrasonography with magnetic resonance imaging using gradient-recalled echo technique in cases of siderosis of spleen was conducted in 53 cirrhotic patients with endoscopic proof of gastroesophageal varices. Of the 34 patients with splenic siderosis on MR imaging, punctate hyperechoic foci were detected in the spleen on ultrasonography in 24 patients. Using MR imaging as the reference standard for the diagnosis of splenic siderosis, the sensitivity of ultrasonography is 70.6%; specificity is 78.9%; positive predictive value is 85.7%; and negative predictive value is 40%. We conclude that ultrasonography is a fairly accurate technique in the diagnosis of splenic siderosis. (+info)
Pathology of the synovium.
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Synovium is specialized mesenchymal tissue that is essential for the appropriate function of the locomotor apparatus. It is the site for a series of pathologic processes that are characteristic, and in some cases specific, to this distinctive tissue. In this article, the normal microscopic anatomy of synovium is briefly reviewed. Synovial proliferative disorders, including pigmented villonodular synovitis, giant cell tumor of tendon sheath, hemosiderotic synovitis, and fatty infiltration of the synovial membrane are discussed. Additionally, the subjects of intrasynovial cartilaginous lesions (primary and secondary synovial chondromatosis) and crystal deposition diseases are reviewed. Finally, the response of synovial tissues to implanted foreign materials that are used in large and small joint arthroplasty are described. (+info)
Hemosiderotic fibrohistiocytic lipomatous lesion: ten cases of a previously undescribed fatty lesion of the foot/ankle.
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We address the clinicopathologic features of a previously undescribed heavily-pigmented spindle cell proliferation within a circumscribed benign lipomatous lesion that occurs mainly in the ankle region of older females. Patients with "lipoma with fibrohistiocytic proliferation" were retrieved from our files. Slides and clinical information were reviewed, and immunohistochemistry was performed (n = 5). Ten patients with hemosiderotic fibrohistiocytic lipomatous lesions were identified. All cases demonstrated a well-circumscribed fatty lesion with random focal proliferations of plump, slightly pleomorphic spindled cells, scattered inflammatory cells, and abundant iron pigment. The spindled cells had vesicular nuclei with indistinct nucleoli; occasional hyperchromatism was observed. No nuclear cytoplasmic inclusions were identified. The spindled component had a reactive appearance. In most cases, the fatty component, with homogeneously sized adipocytes, predominated. The lesions occurred in the foot/ankle region (8/10, one each cheek and hand) of primarily females (8/10) with a mean age of 50.6 years (range 42-63 years), size of 7.7 cm (range 2.5-17 cm), and prior duration of 3.1 years. Seven of eight patients had a history of prior trauma. The spindled component was positive for vimentin, calponin, CD34, and occasionally KP-1 or lysozyme and negative for caldesmon, S100, and desmin. Follow-up on eight patients revealed four with recurrences or residual disease over three years, requiring re-excision. No cases metastasized or caused patient death (mean 12 years, range 1-23 years). We describe a predominantly fatty lesion that is hemosiderin rich with a "fibrohistiocytic" proliferation, composed of histiocytes, myofibroblasts, and C34-positive fibroblasts, which occurs predominantly in the ankle region of middle-aged females. We believe that this is a reactive process due to antecedent trauma, the inflammatory cells, hemosiderin, mixed spindled cells, and homogeneous non-neoplastic appearance of the fat. HFLL can be distinguished from previously described lesions. Correct identification of hemosiderotic fibrohistiocytic lipomatous lesion is important, as it may locally recur. (+info)