Erdheim Chester Disease treated successfully with cladribine
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Erdheim Chester Disease treated successfully with cladribine
A 61-year-old previously healthy male with a history of progressive fatigue, lower extremity edema, and dyspnea for 4 months was hospitalized with pericardial and pleural effusions (Figure 1A, B). Lung, pleural, and pericardial biopsies were consistent with Erdheim-Chester disease. He was treated with systemic steroids, and ultimately tried on PEG-interferon. He deteriorated clinically and the ...
متن کاملCladribine for the Management of Erdheim-Chester Disease in Adults
Erdheim-Chester Disease (ECD) is a rare, non-Langerhans cell histiocytosis characterized by foamy infiltrates of soft tissue and bone, with a histopathology that reveals CD68+, CD1a-, and S100histiocytes densely infiltrating organ systems such as bone, large vessels, heart, and lungs, and other tissues to name a few. Few chemotherapeutic options exist in the second line, of which one is cladrib...
متن کاملErdheim-Chester disease and concomitant tuberculosis successfully treated with chemotherapy and long-term steroids.
Erdheim-Chester disease (ECD) is a rare histiocytosis usually affecting the skeletal system, but visceral organs and central nervous system involvement are common as well. Probability exists that immunomodulatory therapies and disorders can play a role in clinical course of the disease. Because of rarity of the disorder, it is hard to classify it and standardize the treatment options, but, acco...
متن کاملErdheim-Chester Disease
Erdheim-Chester disease (ECD) is a rare, xanthogranulomatous, non-Langerhans cell histiocytosis with frequent systemic involvement. Although the diagnosis is based on characteristic histological and radiological findings, its identification can be challenging because of its heterogeneous presentation. Osteosclerosis of long bones, often associated with bone pain, is the most common initial mani...
متن کاملErdheim-chester disease.
Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis of unknown etiology, characterized by multi-organ involvement. ECD is usually diagnosed on the basis of characteristic radiologic and histopathological findings. Lesions may be skeletal and/or extraskeletal in location, and may include the skin, lung, heart, and central nervous system. We describe here a 68-year-old man ...
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ژورنال
عنوان ژورنال: Respiratory Medicine Case Reports
سال: 2016
ISSN: 2213-0071
DOI: 10.1016/j.rmcr.2016.03.008