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

研究成果: Contribution to journalArticle査読

19 被引用数 (Scopus)

抄録

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.

本文言語英語
ページ(範囲)103-106
ページ数4
ジャーナルPediatric Blood and Cancer
47
1
DOI
出版ステータス出版済み - 7 2006

All Science Journal Classification (ASJC) codes

  • 小児科学、周産期医学および子どもの健康
  • 血液学
  • 腫瘍学

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