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Respiratory Medicine Jan 2021Idiopathic pulmonary hemosiderosis (IPH) is an uncommon cause of diffuse alveolar hemorrhage (DAH). Patients with IPH usually present with hemoptysis, and the diagnosis... (Review)
Review
Idiopathic pulmonary hemosiderosis (IPH) is an uncommon cause of diffuse alveolar hemorrhage (DAH). Patients with IPH usually present with hemoptysis, and the diagnosis is often delayed by years. Patients often present with intermittent episodes of hemoptysis interspersed between periods of relative normalcy. However, massive hemorrhage resulting in acute respiratory failure and non-remitting hemoptysis have also been described. The classic triad includes hemoptysis, radiologic lung infiltrate, and iron deficiency anemia. Several hypotheses regarding the pathogenesis of IPH have been proposed. These risk factors include an autoimmune, allergic or genetic predisposition, and possible environmental exposure. Since IPH appears to be responsive to corticosteroids, the autoimmune hypothesis is considered to play a crucial role. A diagnosis of IPH requires exclusion of other etiologies of DAH, including infection, medications, toxic inhalation, vasculitis, and anti-glomerular basement membrane disease, among others. Histologically, IPH is characterized by the presence of hemosiderin-laden macrophages in the alveolar space without any evidence of vasculitis or immunocomplex deposition. Corticosteroid therapy represents the primary modality of treatment. Other immunosuppressive medications have also been used with varying success, especially in the setting of steroid-refractory disease. The prognosis of IPH in adults is somewhat better compared to the pediatric population. The severity of the initial presentation does not predict future outcomes. Which risk factors and patient characteristics are associated with a poor outcome are also unknown. More research is necessary to elucidate the pathophysiology and appropriate treatment.
Topics: Adrenal Cortex Hormones; Anemia, Iron-Deficiency; Autoimmunity; Child; Delayed Diagnosis; Female; Genetic Predisposition to Disease; Hemoptysis; Hemosiderosis; Humans; Immunosuppressive Agents; Lung; Lung Diseases; Magnetic Resonance Imaging; Male; Middle Aged; Prognosis; Radiography, Thoracic; Risk Factors; Severity of Illness Index; Hemosiderosis, Pulmonary
PubMed: 33246295
DOI: 10.1016/j.rmed.2020.106234 -
JACC. Cardiovascular Imaging Oct 2017Cardiac magnetic resonance (CMR) is a valuable tool for the evaluation of patients with, or at risk for, heart failure and has a growing impact on diagnosis, clinical... (Review)
Review
Cardiac magnetic resonance (CMR) is a valuable tool for the evaluation of patients with, or at risk for, heart failure and has a growing impact on diagnosis, clinical management, and decision making. Through its ability to characterize the myocardium by using multiple different imaging parameters, it provides insight into the etiology of the underlying heart failure and its prognosis. CMR is widely accepted as the reference standard for quantifying chamber size and ejection fraction. Additionally, tissue characterization techniques such as late gadolinium enhancement (LGE) and other quantitative parameters such as T mapping, both native and with measurement of extracellular volume fraction; T mapping; and T* mapping have been validated against histological findings in a wide range of clinical scenarios. In particular, the pattern of LGE in the myocardium can help determine the underlying etiology of the heart failure. The presence and extent of LGE determine prognosis in many of the nonischemic cardiomyopathies. The use of CMR should increase as its utility in characterization and assessment of prognosis in cardiomyopathies is increasingly recognized.
Topics: Amyloidosis; Cardiomyopathies; Cardiomyopathy, Dilated; Cardiomyopathy, Hypertrophic; Contrast Media; Genetic Predisposition to Disease; Hemosiderosis; Humans; Image Interpretation, Computer-Assisted; Magnetic Resonance Imaging; Predictive Value of Tests; Prognosis; Risk Factors; Sarcoidosis; Severity of Illness Index
PubMed: 28982571
DOI: 10.1016/j.jcmg.2017.08.005 -
Respirology Case Reports Aug 2023The co-existence of idiopathic hemosiderosis and celiac disease is Lane-Hamilton Syndrome. This is a rare condition with only a few dozen cases reported to date. Its...
The co-existence of idiopathic hemosiderosis and celiac disease is Lane-Hamilton Syndrome. This is a rare condition with only a few dozen cases reported to date. Its clinical presentation typically involves hemoptysis that can be life-threatening in the acute phase. We report the uncommon case of the development of idiopathic pulmonary hemosiderosis almost a decade after the diagnosis of celiac disease. Delayed diagnosis led to recurrent episodes of large volume hemoptysis despite immunosuppressive therapy due to ongoing ingestion of gluten. High doses of glucocorticoids accompanied by a cell cycle inhibitor mycophenolate mofetil were required for treatment. A strict gluten free diet is vital to control the disease. We highlight the importance of identifying this syndrome and definitive treatment, including avoidance of dietary triggers in addition to conventional immunosuppressive therapy.
PubMed: 37416498
DOI: 10.1002/rcr2.1188 -
The British Journal of Radiology Mar 2019This article highlights the range of osseous findings that can be encountered as well as the imaging features of extramedullary haematopoiesis. As iron overload remains... (Review)
Review
This article highlights the range of osseous findings that can be encountered as well as the imaging features of extramedullary haematopoiesis. As iron overload remains a major cause of morbidity and mortality in these disorders, we also discuss the MRI evaluation of hepatic and cardiac hemosiderosis, to aid in the optimization of iron chelation therapy. Future imaging use will be dictated by evolving clinical needs, such as in screening for emerging morbidities, including hepatic fibrosis and hepatocellular carcinoma.
Topics: Bone Diseases; Heart; Hematopoiesis, Extramedullary; Hemosiderosis; Humans; Liver; Magnetic Resonance Imaging; Thalassemia
PubMed: 30412423
DOI: 10.1259/bjr.20180658 -
Anais Brasileiros de Dermatologia 2017We report a rare clinical case of hemosiderotic dermatofibroma in a 36-year-old female patient. The main dermatoscopic finding was represented by homogeneous blue-gray...
We report a rare clinical case of hemosiderotic dermatofibroma in a 36-year-old female patient. The main dermatoscopic finding was represented by homogeneous blue-gray pigmentation. The aim of this report is to demonstrate the rarity of the lesion and the dermatoscopic importance it assumes by sharing a blue-gray homogeneous pattern with other benign and malignant lesions.
Topics: Adult; Diagnosis, Differential; Female; Hemosiderosis; Humans; Melanoma; Skin Neoplasms
PubMed: 28225963
DOI: 10.1590/abd1806-4841.20173563 -
Case Reports in Pediatrics 2014Idiopathic pulmonary hemosiderosis (IPH) is a rare complex entity characterized clinically by acute or recurrent episodes of hemoptysis secondary to diffuse alveolar...
Idiopathic pulmonary hemosiderosis (IPH) is a rare complex entity characterized clinically by acute or recurrent episodes of hemoptysis secondary to diffuse alveolar hemorrhage. The radiographic features are variable, including diffuse alveolar-type infiltrates, and interstitial reticular and micronodular patterns. We describe a 3-week-old infant presenting with hemoptysis and moderate respiratory distress. Idiopathic pulmonary hemosiderosis was the first working diagnosis at the Emergency Department and was confirmed, 2 weeks later, by histological studies (bronchoalveolar lavage). The immunosuppressive therapy by 1 mg/kg/d prednisone was immediately started, the baby returned home on steroid therapy at a dose of 0,5 mg/kg/d. The diagnosis of idiopathic pulmonary hemosiderosis should be evocated at any age, even in the neonate, when the clinical presentation (hemoptysis and abnormal radiological chest images) is strongly suggestive.
PubMed: 25389504
DOI: 10.1155/2014/463973 -
Archives of Pathology & Laboratory... Nov 2017Myxoinflammatory fibroblastic sarcoma is a rare soft tissue tumor with most occurring in the distal extremities of adult patients. It has a high rate of local recurrence... (Review)
Review
Myxoinflammatory fibroblastic sarcoma is a rare soft tissue tumor with most occurring in the distal extremities of adult patients. It has a high rate of local recurrence and a low rate of metastasis. Because it may appear benign on clinical examination, and because the microscopic features are generally underrecognized, it is often inadequately treated and misdiagnosed. In this review, based upon experience and that of the literature, the intent is to highlight salient clinicopathologic features, detail the broad microscopic spectrum including high-grade aggressive variants, review the molecular features, and discuss its relation to hemosiderotic fibrolipomatous tumor.
Topics: Diagnosis, Differential; Emperipolesis; Extremities; Fibrosarcoma; Hemosiderosis; Humans; Lipoma; Myxosarcoma; Neoplasm Recurrence, Local; Neoplasms, Fibrous Tissue; Prognosis
PubMed: 29072951
DOI: 10.5858/arpa.2017-0219-RA -
Neurology India 2020
Topics: Brain; Hemosiderosis; Humans
PubMed: 33342913
DOI: 10.4103/0028-3886.304114