Cases reported "Hemosiderosis"

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1/17. Pulmonary haemosiderosis in Saudi children: report of two cases and review of the literature.

    Two children with idiopathic pulmonary haemosiderosis are reported. These are the first cases of this disease from saudi arabia and highlight the diagnostic problem caused by their presentation as cases of iron deficiency anaemia. The aetiopathogenesis, clinical presentation, diagnosis and management are discussed. The attention of paediatricians is drawn to this relatively rare cause of iron deficiency anaemia.
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keywords = anaemia
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2/17. Idiopathic pulmonary haemosiderosis.

    Idiopathic pulmonary haemosiderosis (IPH) is a rare disorder characterized by recurrent bouts of haemoptysis, iron deficiency anaemia, and pulmonary infiltrates on chest radiograph. We present such a case and believe that it is the first report from the middle east.
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keywords = anaemia
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3/17. 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 = anaemia
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4/17. 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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ranking = 2.5
keywords = anaemia
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5/17. Pitfalls in the diagnosis of idiopathic pulmonary haemosiderosis.

    Idiopathic pulmonary haemosiderosis is a very rare but devastating disorder. diagnosis is sometimes difficult and the clinical course exceedingly variable, as illustrated by this report of a girl, aged 2 years 4 months, with severe iron deficiency anaemia. There was no response to iron therapy and transfusions. Sustained and striking reticulocytosis associated with low haptoglobin mimicked haemolytic anaemia. Positive faecal blood test was documented after repeated testing. There were no pulmonary symptoms. A chest radiograph showed bilateral diffuse alveolar infiltrates. bronchoalveolar lavage fluid showed numerous siderophages. High resolution computed tomography of the thorax revealed early pulmonary fibrotic changes. Recurrent reticulocytosis appeared to be a very useful sign of recurrent bleeding episodes.
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keywords = anaemia
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6/17. 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 = anaemia
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7/17. Coeliac disease as a cause of unusually severe anaemia in a young man with idiopathic pulmonary haemosiderosis.

    Idiopathic pulmonary haemosiderosis (IPH) is characterized by a triad of recurrent episodes of alveolar haemorrhage, haemoptysis and iron deficiency anaemia. The combination of IPH and coeliac disease (CD) is extremely rare though both diseases may have a common pathogenetic link. As illustrated by our case CD should be specifically looked for in patients with IPH, especially those in whom the severity of anaemia is disproportionate to radiologic findings even in the absence of gastrointestinal symptoms since both diseases may benefit from a gluten-free diet.
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ranking = 3
keywords = anaemia
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8/17. Transfusion haemosiderosis inspite of regular use of desferrioxamine: case report.

    We describe a case of a female who developed haemosiderosis, in the course of treatment for very severe unstable aplastic anaemia for fourteen years. She was 37 years old at the time of initial diagnosis. Her management consisted of regular blood transfusions aimed at haemoglobin above 8.5 g/dl, antimicrobials, oxymetholone, low dose prednisone and folate. She had received about seventy five units of blood at the start of 2 grams of desferrioxamine with every subsequent blood transfusion. Annual tests of serum ferritin showed progressive increase. She developed skin changes, diabetes mellitus, heart disease, recurrent infections, generalized joint and abdominal pains and liver failure. She died within six weeks of developing congestive heart failure coupled with liver failure due to haemosiderosis despite regular use of desferrioxamine.
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9/17. Idiopathic pulmonary haemosiderosis: two case reports.

    Idiopathic pulmonary haemosiderosis (IPH) is a rare disorder and is characterised by the occurrence of episodes of diffuse alveolar haemorrhage (DAH). The patients usually present with a history of recurrent haemoptysis, anaemia and alveolar opacities on chest radiograph. The radiographic features are very characteristic and reveal alveolar opacities in middle and lower zones with selective sparing of the upper zones. An extensive diagnostic work-up is needed to exclude other causes of DAH. We report two cases of idiopathic pulmonary haemosiderosis who responded well to treatment with oral glucocorticoids.
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keywords = anaemia
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10/17. Congenital dyserythropoietic anaemia (type II) presenting with haemosiderosis.

    A 39-year-old female with type II congenital dyserythropoiesis presented with iron overload. The clinical and haematologic features were an anaemia of variable severity, splenomegaly, numerous bizarre and binucleate normoblasts in the bone marrow, with prominent submembranous cisternae in the late forms, a positive Ham's acid lysis test and aberrant expression of the I and i red cell antigens. The iron overload resulted from gross ineffective erythropoiesis, with accelerated plasma iron turnover and increased absorption aggravated by inappropriate replacement therapy for past episodes of anaemia.
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ranking = 3
keywords = anaemia
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