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Inflammatory, Hypersensitivity and Immune Lung Diseases, including Parasitic Diseases.
Published in Fred W Wright, Radiology of the Chest and Related Conditions, 2022
Many patients with idiopathic haemosiderosis are anaemic and therapeutic removal of blood is therefore impracticable; patients with this tend to have a slow relentless downhill course with increasing breathlessness lasting 12 to 24 months.
Correlation between ferritin levels and Peak Expiratory Flow (PEF) value in thalassemia patients at a private hospital in Indonesia in 2018
Published in Ade Gafar Abdullah, Isma Widiaty, Cep Ubad Abdullah, Medical Technology and Environmental Health, 2020
Y.D. Suryani, W. Risakti, R.G. Ibnusantosa
The results of this study show no relationship between ferritin levels and lung function in thalassemia patients at Al-Ihsan Regional Hospital. The respondents’ age factor and the use of chelating agents can influence the results of the study. The use of chelating agents is considered effective for maintaining iron levels in the body. Although it does not reduce iron to normal levels, it can reduce the likelihood of complications of hemosiderosis. Respondents in this study consumed chelating agents early and regularly, so they could minimize the possibility of hemosiderosis in the lungs that can cause fibrosis.
Sideroblastic Anemia and Porphyrias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Patients with idiopathic sideroblastic anemia may be asymptomatic and not require blood transfusions. Other symptomatic patients require blood transfusions at regular intervals. In these individuals, repeated transfusions can lead to hemosiderosis. Some require an iron chelating agent, such as desferrioxamine, for removal of excessive amounts of iron. Certain individuals respond rapidly and dramatically to desferrioxamine, with a fall in ferritin levels to the normal range. In these patients, there may be a diminution or even a temporary cessation of blood transfusions. Various hematinics such as folic acid and pyridoxine have been tried alone or in combination. For the most part these agents are ineffective, and many patients are transfusion dependent (Table 2).
An Autopsy Case of β-Thalassemia Major Illuminating the Pathological Spectrum of the Disease
Published in Hemoglobin, 2021
Ridhi Sood, Pulkit Rastogi, Deepak Bansal, Reena Das, Prashant Sharma, Geethanjali Gude, Mukesh Dhankar
According to a US-based study, mortality in chronically transfused thalassemia patients is three times that of the general population [6]. Complications, which ultimately lead to death in thalassemia can be multifactorial and depend on the mutation type, phenotypic modifiers and therapy adherence. The etiology of the complications can be the primary pathology, that is, ineffective and extramedullary hematopoiesis or/and treatment-related side effects such as transfusion-derived iron overload and associated infections. The discussed index patient was transfusion-dependent and had a serum ferritin value of <1000.0 ng/dL. Despite this, extensive hemosiderosis was demonstrated in the heart, liver, pancreas, spleen, and endocrine organs on autopsy examination. This again reiterates that serum ferritin is not a good indicator of iron overload, and more sophisticated modalities are needed to assess it [7,8]. The HFE and HJV gene mutations were not detected, and they have been previously shown by our group to be extremely rare among Indians [9].
Compliance with Deferoxamine Therapy and Thyroid Dysfunction of Patients with β-Thalassemia Major in Syria
Published in Hemoglobin, 2019
M. Yousuf Yassouf, Faizeh Alquobaili, Younes Kabalan, Yasser Mukhalalaty
β-Thalassemias are autosomal recessive inherited disorders characterized by reduced production of adult hemoglobin (Hb), resulting from the reduced synthesis of β-globin chains, thus causing abnormal erythropoiesis. β-Thalassemia major (β-TM), is the severe, transfusion-dependent form of β-thalassemia (β-thal) [1]. High frequency of this disease is found in the Mediterranean Basin, the Middle East, Southeast Asia, the Indian subcontinent and Africa [2]. In Syria, β-thal trait prevalence is expected to be about 6.0% of the population, with an estimated 779,000 carriers, and more than 8000 registered transfusion-dependent patients [3,4]. Standard treatment for patients with β-TM includes regular blood transfusions and chelation therapy. Nevertheless, total body iron load gradually increases because of repeated transfusions and increased intestinal iron absorption, which leads to subsequent hemosiderosis with cardiac, endocrine and hepatic complications [5].
Iron pill pneumonitis with reactive lymph nodes
Published in Baylor University Medical Center Proceedings, 2018
Letlhogonolo Tlhabano, Rohan Mankikar, Chibuzo Odigwe, William Owens, Lawrence Grant, Rohan Arya
Pathology showed fibrosing/granulomatous tissue with prominent hemosiderosis without evidence of malignancy (Figure 2). Bronchoalveolar lavage was negative for malignancy, and all cultures were negative. There was still significant concern for a malignant process, so the patient underwent bronchoscopy with endobronchial ultrasound-guided biopsy of the subcarinal lymph node. Cytology did not reveal any malignancy; a cell block revealed nonnecrotizing granulomas. The lymph node biopsy was stained for acid-fast bacilli and for fungus using Gomori methenamine silver, and both stains were negative.