Isolated juvenile xanthogranuloma in the bone marrow: report of a case and review of the literature.

Chimen Kesserwan, Daniel R Boué, Samir B Kahwash

Journal: Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society 2007;10(2):161-4

PMID: 17378689

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Abstract

We report a case of juvenile xanthogranuloma limited to involvement of the bone marrow in a 6-week-old male infant. Evaluation of the bone marrow was a part of the workup for peripheral blood cytopenia. Examination showed hypercellular marrow with paratrabecular clusters of lipidized histiocytes positive for CD68, CD4, and factor XIII(a) and negative for S100 and CD1a. Clinical and radiological workup showed no associated skin lesions or osseous or visceral involvement. The patient was started on chemotherapy with clinical improvement and gradual decreased bone marrow involvement. The child is alive and well at 16 months of age. This case represents, to the best of our knowledge, the 1st documented case of juvenile xanthogranuloma with isolated bone marrow involvement sparing skin and viscera.

Address: Department of Laboratory Medicine, Children's Hospital, 700 Children's Drive, Columbus, OH 43205, USA.

DOI: 10.2350/06-06-0106.1

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MeSH Terms: Antigens, CD, Antineoplastic Agents, Phytogenic, Biopsy, Bone Marrow, Factor XIIIa, Follow-Up Studies, Humans, Immunohistochemistry, Infant, Lipopolysaccharide Receptors, Male, Radiography, Receptors, Complement 3b, Time Factors, Treatment Outcome, Vimentin, Vincristine, Xanthogranuloma, Juvenile