Prolonged severe pancytopenia preceding the cutaneous lesions of juvenile xanthogranuloma

Takuya Hara; Shouichi Ohga; Sagano Hattori; Miho Hatano; Noriyuki Kaku; Akihiko Nomura; Hidetoshi Takada; Hisashi Kokuba; Koichi Ohshima; Toshiro Hara

doi:10.1002/pbc.20514

Prolonged severe pancytopenia preceding the cutaneous lesions of juvenile xanthogranuloma

Takuya Hara, Shouichi Ohga, Sagano Hattori, Miho Hatano, Noriyuki Kaku, Akihiko Nomura, Hidetoshi Takada, Hisashi Kokuba, Koichi Ohshima, Toshiro Hara

Faculty of Medical Sciences

Research output: Contribution to journal › Article › peer-review

18 Citations (Scopus)

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 JXC 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.

Original language	English
Pages (from-to)	103-106
Number of pages	4
Journal	Pediatric Blood and Cancer
Volume	47
Issue number	1
DOIs	https://doi.org/10.1002/pbc.20514
Publication status	Published - Jul 2006

All Science Journal Classification (ASJC) codes

Pediatrics, Perinatology, and Child Health
Hematology
Oncology

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10.1002/pbc.20514

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Prolonged severe pancytopenia preceding the cutaneous lesions of juvenile xanthogranuloma. / Hara, Takuya; Ohga, Shouichi; Hattori, Sagano; Hatano, Miho; Kaku, Noriyuki; Nomura, Akihiko; Takada, Hidetoshi; Kokuba, Hisashi; Ohshima, Koichi; Hara, Toshiro.

In: Pediatric Blood and Cancer, Vol. 47, No. 1, 07.2006, p. 103-106.

Research output: Contribution to journal › Article › peer-review

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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 JXC 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.",

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AU - Kaku, Noriyuki

AU - Nomura, Akihiko

AU - Takada, Hidetoshi

AU - Kokuba, Hisashi

AU - Ohshima, Koichi

AU - Hara, Toshiro

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