Treatment of Erdheim-Chester disease with cladribine: a rational approach

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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 ...

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Cladribine 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...

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Erdheim-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...

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Erdheim-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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Erdheim-Chester disease.

A new case of Erdheim-Chester lipoid granulomatosis is described in a 40-year-old man. In this lipid storage disease, characteristic symmetrical osteosclerotic areas are observed mainly in metadiaphyses of long tubular bones. Bone biopsy is mandatory for definite diagnosis, although characteristic radiographic pictures often provide an important clue to the correct diagnosis.

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ژورنال

عنوان ژورنال: British Journal of Ophthalmology

سال: 2004

ISSN: 0007-1161

DOI: 10.1136/bjo.2003.035584