title: Prolonged severe pancytopenia preceding the cutaneous lesions of juvenile xanthogranuloma.
abstract: We report a case of juvenile xanthogranuloma (JXG) having progressive pancytopenia for 6 months until the proliferating skin lesions. A 2-month-old infant presented recurrent fever, anemia, and hepatosplenomegaly mimicking hemophagocytic lymphohistiocytosis (HLH) or juvenile myelomonocytic leukemia (JMML). At 8 months of age, the biopsy of a growing papule on the elbow made the diagnosis. Bone marrow (BM) specimens showed clustering foamy cells including hemophagocytosis by histiocytes. Treatment with etoposide followed by vinblastine plus prednisolone (PSL) therapy improved the disease. Although JXG is a benign non-Langerhans cell histiocytosis, the multisystem-visceral form should be considered as a potential aggressive disease when associated with BM failure in early infancy.
authors.affiliation: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Maidashi, Fukuoka, Japan.
authors.forename: Takuya
authors.initials: T
authors.lastname: Hara
authors.forename: Shouichi
authors.initials: S
authors.lastname: Ohga
authors.forename: Sagano
authors.initials: S
authors.lastname: Hattori
authors.forename: Miho
authors.initials: M
authors.lastname: Hatano
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Kaku
authors.forename: Akihiko
authors.initials: A
authors.lastname: Nomura
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Takada
authors.forename: Hisashi
authors.initials: H
authors.lastname: Kokuba
authors.forename: Koichi
authors.initials: K
authors.lastname: Ohshima
authors.forename: Toshiro
authors.initials: T
authors.lastname: Hara
mesh_terms: D001778:Blood Coagulation Disorders / Q000209:etiology; D001853:Bone Marrow / Q000473:pathology; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D007223:Infant; D008297:Male; D010198:Pancytopenia / Q000209:etiology*; D013163:Splenomegaly / Q000209:etiology; D014972:Xanthogranuloma, Juvenile / Q000150:complications* / Q000473:pathology*