Successful treatment of systemic juvenile xanthogranulomatosis with cytarabine and 2-chlorodeoxyadenosine: case report and review of the literature

Abstract

The non-Langerhans cell histiocytosis juvenile xanthogranulomatosis (JXG) is usually a benign disease limited to the skin. Only few cases of systemic disease with ≥2 affected organs and lethal outcome have been reported yet. Treatment is controversial and no standard protocol is available. We report the rare case of a 22-month-old boy presenting multiple erythematous-brownish papules of head, trunk and legs which had developed starting from his sixth′ month of life. Additional symptoms were delayed psychomotoric development, hydrocephalus and hepatosplenomegaly. Further diagnostics revealed a systemic JXG with involvement of skin, central nervous system, liver and spleen. The patient did not respond to initial therapy with prednisone and vinblastin according to protocol III for Langerhans cell histiocytosis. However, further therapy with cytarabine and 2-chlorodeoxyadenosine followed by a consolidation phase with 2-chlorodeoxyadenosine alone was successful and the patient is in his 4th year in remission. We provide a comprehensive review of the reported cases with systemic JXG to date.

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