Bone marrow findings in Erdheim-Chester disease: increased prevalence of chronic myeloid neoplasms
نویسندگان
چکیده
منابع مشابه
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...
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 ...
متن کاملErdheim-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.
متن کاملGastrointestinal Erdheim-Chester disease.
We report a rare case of Erdheim-Chester Disease, a non-Langerhans cell histiocytosis. A 60-year old female presented with a seven-month history of vague abdominal symptoms. A large retroperitoneal mass was detected on computed tomography (CT), but multiple CT-guided biopsy samples were inconclusive. Laparoscopy revealed a mass in the distal ileum, which was resected. Histology and immuno-histo...
متن کاملErdheim-chester Disease
DEFINITION The histopathologic characteristics of ECD overlap xanthogranuloma and distinctions between the two are made on clinical and radiologic findings. Lipid-laden histocytes with foamy or eosinophilic cytoplasm infiltrate bones and various organs and generate a fibroblastic response that leads to critical organ failure. The histocytes are CD68+, Factor XIIIa+ CD1a-, S100-, and lack Birbec...
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ژورنال
عنوان ژورنال: Haematologica
سال: 2019
ISSN: 0390-6078,1592-8721
DOI: 10.3324/haematol.2019.234187