Abstract |
Systemic form of juvenile xanthogranuloma with involvement of liver and bone marrow is reported in a 2-month-old female infant who presented with hepatosplenomegaly, severe anemia, and thrombocytopenia. There was no skin lesion, nor bone lesion. The enlarged liver has generalized yellowish spots. The diagnosis of juvenile xanthogranuloma was made by pathologic findings of marrow and portal tract infiltration by S-100 negative, CD1a negative, CD68 positive, and Factor XIIIa positive large pale to foamy histiocytes with Touton giant cells, and lack of Langerhans cell granule by electron microscopic examination. The patient was treated with Vinblastine and Etoposide, and experienced slow and gradual disease regression in one year. To the best of knowledge, this is the first documented case of bone marrow involvement in systemic juvenile xanthogranuloma.
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Authors | Chavit Chantranuwat |
Journal | Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
(Pediatr Dev Pathol)
2004 Nov-Dec
Vol. 7
Issue 6
Pg. 646-8
ISSN: 1093-5266 [Print] United States |
PMID | 15630537
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Antineoplastic Agents, Phytogenic
- Vinblastine
- Etoposide
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Topics |
- Antineoplastic Agents, Phytogenic
(therapeutic use)
- Bone Marrow
(pathology)
- Etoposide
(therapeutic use)
- Female
- Humans
- Infant
- Liver
(pathology)
- Treatment Outcome
- Vinblastine
(therapeutic use)
- Xanthogranuloma, Juvenile
(drug therapy, pathology)
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