Cases reported "Hemosiderosis"

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1/35. Microvesicular steatosis, hemosiderosis and rapid development of liver cirrhosis in a patient with Pearson's syndrome.

    BACKGROUND/AIMS: Pearson's marrow-pancreas syndrome consists of refractory sideroblastic anemia with vacuolization of marrow precursors and exocrine pancreas dysfunction. patients with this disease usually have large deletions of the mitochondrial genome. We report a patient with Pearson's syndrome who had predominantly hepatic manifestations such as microvesicular steatosis, hemosiderosis and rapidly developing cirrhosis. methods: Analysis of the mitochondrial and nuclear genomes, determination of enzyme activities and of the hepatic iron content were performed using standard techniques of molecular biology and biochemistry. RESULTS: The patient had typical ringed sideroblasts in a bone marrow smear and a 7436-bp deletion of the mitochondrial genome in all tissues investigated, compatible with Pearson's syndrome. He died within 3 months after birth due to liver failure. Histopathological analysis of the liver revealed complete cirrhosis with signs of chronic cholestasis, microvesicular steatosis and massive hemosiderosis. In addition, the patient was heterozygous for the C282Y and H63D mutations of the hemochromatosis gene. CONCLUSIONS: Pearson's syndrome should be added to the list of neonatal diseases which can cause microvesicular steatosis, hepatic accumulation of iron and liver cirrhosis.
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2/35. bone marrow transplantation for aplastic anaemia from a HL-A and MLC-identical unrelated donor.

    Bone-marrow transplantation (BMT) from an unrelated, HL-A-phenotype-identical, MLC-negative donor was performed in a 31 year old woman with severe longlasting aplastic anemia. in vitro assays failed to demonstrate humoral or cellular sensitization of the recipient against donor-type antigens. Following conditioning with cyclophosphamide, prompt but only transient engraftment of the transplant occurred accompanied by signs of mild graft-versus-host-disease (GVHD) of the liver. The results of a second bone marrow transplantation from the same donor cannot be evaluated due to early death of the recipient. It is concluded that bone marrow from unrelated, HL-A and MLC-identical donors may engraft without severe GVHD. Rejection of the graft in our patient may have been related to greater antigenic differences that can be expected to exist between HL-A and MLC-identical unrelated individuals than between HL-A and MLC-identical siblings. However, insufficient preparative immunosuppression with cyclophosphamide due to severe hepatic hemosiderosis appears equally likely as the cause of graft rejection. The possibly increased risk of graft rejection or severe GVHD should not preclude the use of unrelated HL-A and MLC-identical marrow donors, when histocompatible sibling donors are not available; but more potent immunosuppressive regimens than the cyclophosphamide protocol may be necessary to ensure permanent engraftment.
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keywords = bone
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3/35. Idiopathic pulmonary hemosiderosis in a child: report of one case.

    Idiopathic pulmonary hemosiderosis (IPH) is uncommon in children. We report a 3-year-old girl who was presented with acute pale-looking appearance, hemoptysis, hematemesis and shortness of breath. This patient was confirmed to have pulmonary hemorrhage by the presence of hemosiderin-laden macrophages in the bronchoalveolar lavage fluid using a flexible bronchoscope. Other causes of PH including glomerular, cardiac and immunological disorder were excluded by normal laboratory studies. She was primarily treated by oral prednisolone, but due to recurrent hemoptysis, immunosuppressive agent was added for maintenance therapy. Pediatricians should consider PH in a patient who has recurrent dyspnea, hemoptysis and iron deficiency anemia.
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keywords = macrophage
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4/35. Idiopathic pulmonary hemosiderosis in an adult. Favourable response to azathioprine.

    Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder characterised by intermittent, diffuse alveolar hemorrhage (DAH). Although an inflammatory pulmonary capillaritis can be evidenced in most patients with DAH, IPH is a distinct entity in which pulmonary inflammatory alterations are lacking. Most cases occur in children, although the disease has been exceptionally reported in adults too. Here, we, describe a case of IPH in a 30-year-old woman who was admitted to our hospital because of recurrent episodes of hemoptysis since the age of 21. IPH was diagnosed on the basis of: 1) an open lung biopsy showing focal alveolar edema and hemorrhage without parenchymal inflammatory alterations, 2) a bronchoalveolar lavage showing hemosiderin-laden macrophages, and 3) exclusion of infectious or immunologic causes of hemoptysis. prednisone administration could control the disease, but every attempt to lower the dose to less than 25 mg per day was followed by recurrence of hemoptysis. Then, azathioprine was started, and after three months prednisone was gradually tapered to the dose of 10 mg per day, without any relapse of the disease. These findings indicate that azathioprine, in combination with prednisone, may be an effective therapy for IPH and suggest that an immunologic mechanism could be involved in the pulmonary capillary damage underlying alveolar bleeding.
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5/35. mitral valve prolapse with pulmonary haemosiderosis and severe anaemia: cause or association?

    A 12 years boy presented with the history of pallor for one month. Two days before hospitalisation he developed fever, cough, shortness of breath. He had past history of such episode.On examination, his heart rate was found to be 120/minute, respiratory rate 40/minute and moderate anaemia was detected. Scattered creptus was audible over mid and lower lung fields and a soft systolic murmur was auscultated at apex. On investigations, Hb was found as 4.6 g/dl and HbF was less than 2%. plasma Hb was 5 g/dl. Straight x-ray chest showed bilateral patchy opacities over mid and lower zones. His sputum was found to be blood stained and prompted the possibility of blood loss occurring in the lungs, which was confirmed by demonstrating haemosiderin laden macrophages on three consecutive sputum specimens. echocardiography revealed a systolic displacement of mitral valve leaflets into the left atrium with co-optation superior to the plane of mitral annulus. Doppler study showed a minimal late systolic regurgitation. It was decided to treat the case as idiopathic pulmonary haemosiderosis. oxygen inhalation, hypertonic saline nebulisation, i.v. hydrocortisone, packed cell transfusion followed by oral prednisolone improved the patient's condition. After 3 months of discontinuing prednisolone, he remained asymptomatic. Here one case of pulmonary haemosiderosis characterised by abnormal accumulation of haemosiderin in the lungs following repeated alveolar haemorrhages with the presence of mitral valve prolapse is reported.
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keywords = macrophage
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6/35. Severe iron deficiency anemia in a child with idiopathic pulmonary hemosiderosis: a case report.

    We report a case of idiopathic pulmonary hemosiderosis (IPH) in a three-year-old male patient who presented with severe iron deficiency anemia. The child had been diagnosed with iron deficiency anemia nine months earlier and had received multiple blood transfusions, but the cause of his anemia had not been established. The diagnosis of IPH was made after a biopsy of the left lung showed large numbers of hemosiderin-filled macrophages in the alveoli. He did not respond to standard dose corticosteroid (CS) treatment (2 mg/kg/d). However, high-dose short-term CS treatment was successful in two episodes of acute respiratory hemorrhage in this patient. We conclude that IPH should always be considered when investigating the cause of iron deficiency anemia. A more rapid diagnosis in this case could have prevented unnecessary investigations and blood transfusions. We also suggest that high-dose short-term CS treatment should be kept in mind, especially in patients who do not respond to a standard dose.
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ranking = 11573.530691753
keywords = macrophage
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7/35. Pediatric idiopathic pulmonary hemosiderosis diagnosed by sputum analysis: plain radiography and computed tomography findings.

    OBJECTIVE: Idiopathic pulmonary hemosiderosis (IPH) is an uncommon disorder, which is characterized by recurrent hemoptysis, iron deficiency anemia and diffuse parenchymal infiltration on chest radiographs in pediatric patients. We wish to present clinical and radiological (plain radiography and CT) findings of this rare pathology. CLINICAL PRESENTATION AND INTERVENTION: A 14-year-old girl was admitted to the pediatric emergency department with complaints of cough, dyspnea, fatigue and bloody sputum for 6 months. She had been hospitalized 3 times during this period and received antibiotics and blood transfusion. Chest x-rays revealed prominent perihilar and bibasilar consolidation. CT showed a ground glass pattern and consolidated areas with increased density. sputum analysis yielded hemosiderin-laden macrophages. With presumptive diagnosis of IPH, prednisolone was administered. Her symptoms improved on the 5th day of treatment and 1 month later, plain chest radiography demonstrated marked improvement. CONCLUSION: Although IPH is a rare condition, the diagnosis of IPH should be considered, among others, in a patient with hemoptysis and bilateral infiltration in the chest X-ray. This may prevent antibiotic misuse and risk of death due to severe hemorrhage.
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keywords = macrophage
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8/35. Localized hemosiderosis as a sequela of acne.

    Dark blue-black pigmentation has persisted in areas of previous acne scarring in a young woman. By histologic and electron microscopic examination, the pigment is demonstrated to represent hemosiderin. Much of the hemosiderin is lying free within macrophages, not bound by lysosomal membranes that normally would protect the cell from this toxic material.
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keywords = macrophage
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9/35. Idiopathic pulmonary haemosiderosis revisited.

    Idiopathic pulmonary haemosiderosis is a rare cause of diffuse alveolar haemorrhage of unknown aetiology. It occurs most frequently in children, has a variable natural history with repetitive episodes of diffuse alveolar haemorrhage, and has been reported to have a high mortality. Many patients develop iron deficiency anaemia secondary to deposition of haemosiderin iron in the alveoli. Examination of sputum and bronchoalveolar lavage fluid can disclose haemosiderin-laden alveolar macrophages (siderophages), and the lung biopsy shows numerous siderophages in the alveoli, without any evidence of pulmonary vasculitis, nonspecific/granulomatous inflammation, or deposition of immunoglobulins. Contrary to earlier reports, corticosteroids alone or in combination with other immunosuppressive agents may be effective for either exacerbations or maintenance therapy of idiopathic pulmonary haemosiderosis.
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keywords = macrophage
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10/35. anemia as the sole presenting symptom of idiopathic pulmonary hemosiderosis: report of two cases.

    Idiopathic pulmonary hemosiderosis (IPH) is a rare disease in children and has an unknown etiology. It is characterized by the triad of hemoptysis, pulmonary infiltrates on chest radiograph (CXR) and iron deficiency anemia. We report two young children, aged 3 and 4 years, were admitted due to pale-looking appearance but without hemoptysis or other respiratory symptoms. pallor was the sole presenting feature in these 2 children with IPH and which was unusual. CXR obtained on admission led to the suspicion of pulmonary hemorrhage. The diagnosis of IPH was confirmed based on the presence of many hemosiderin-laden macrophages in bronchoalveolar lavage fluid obtained by flexible bronchoscopy. Steroid was initiated after the diagnosis of IPH was established; the both of them have been well and received regular follow-up in our outpatient department. IPH may not be diagnosed because of difficulty in diagnosis. anemia may be the only presenting feature of IPH, which was due to occult pulmonary hemorrhage. Initial treatment with corticosteroids has been successful in our patients for a period of 6 and 8 months of follow up respectively.
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keywords = macrophage
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