Rare association of juvenile xanthogranuloma and acute lymphoblastic leukemia

Abstract

Juvenile xanthogranuloma (JXG) is a rare non-Langerhans cell histiocytosis that usually occurs during infancy and early childhood. It is seen in combination with juvenile chronic myelomonocytic leukemia and/or neurofibromatosis type 1. The association with acute lymphoblastic leukemia (ALL) is rarely seen. We present a 2-year-old boy who had this rare combination and presented with multiple tan-orange-colored macules, papules, and nodules over the upper body and distal aspect of lower limbs for 4 months of age. The histologic evaluation and immunohistochemistry analysis resulted in the diagnosis of JXG. Bone marrow biopsy showed lymphoblastosis with marked suppression of erythroid and myeloid cells, suggesting ALL.

Keywords

• acute lymphoblastic leukemia
• juvenile xanthogranuloma
• non-langerhans cell histiocytosis