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Prolonged severe pancytopenia preceding the cutaneous lesions of juvenile xanthogranuloma
Author(s) -
Hara Takuya,
Ohga Shouichi,
Hattori Sagano,
Hatano Miho,
Kaku Noriyuki,
Nomura Akihiko,
Takada Hidetoshi,
Kokuba Hisashi,
Ohshima Koichi,
Hara Toshiro
Publication year - 2006
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.20514
Subject(s) - medicine , pancytopenia , juvenile xanthogranuloma , hepatosplenomegaly , hemophagocytosis , langerhans cell histiocytosis , hemophagocytic lymphohistiocytosis , histiocytosis , pathology , histiocyte , bone marrow , prednisolone , juvenile myelomonocytic leukemia , dermatology , disease , surgery , stem cell , haematopoiesis , biology , genetics
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. © 2005 Wiley‐Liss, Inc.