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Unusual presentation of Erdheim-Chester disease in a child with acute lymphoblastic leukemia
Archana George Vallonthaiel1, Asit Ranjan Mridha1, Shivanand Gamanagatti1
1Archana George Vallonthaiel, Asit Ranjan Mridha, Mehar Chand Sharma, Department of Pathology, All India Institute of Medical Sciences, New Delhi 110029, India.
Abstract:
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.
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