Open AccessUnusual presentation of Erdheim-Chester disease in a child with acute lymphoblastic leukemia
Author(s) -
Archana George Vallonthaiel,
Asit Ranjan Mridha,
Shivanand Gamanagatti,
Manisha Jana,
Mehar Chand Sharma,
Shah Alam Khan,
Sameer Bakhshi
Publication year - 2016
Publication title -
world journal of radiology
Language(s) - English
Resource type - Journals
ISSN - 1949-8470
DOI - 10.4329/wjr.v8.i8.757
Subject(s) - medicine , osteosclerosis , erdheim–chester disease , pathology , lesion , histiocytosis , langerhans cell histiocytosis , presentation (obstetrics) , radiology , disease
Erdheim-Chester disease (ECD) is an uncommon, non-familial, non-Langerhans cell histiocytosis, which involves skeletal system and soft tissue usually in middle aged and elderly patients. The characteristic radiologic features include bilateral, symmetric cortical osteosclerosis of the diaphyseal and metaphyseal parts of the long bones, or bilateral symmetrically abnormal intense (99m)Technetium labelling of the metaphyseal-diaphyseal region of the long bones, and computed tomography scan findings of "coated aorta" or "hairy kidneys". ECD in childhood with osteolytic lesion is extremely rare. We describe an unusual case with an expansile lytic bone lesion at presentation in a case of acute lymphoblastic leukemia.