日本未熟児新生児学会雑誌　第22巻　第1号　97～103頁（2010年）

日本未熟児新生児学会雑誌　22(1)：97-103；2010　

受付日：平成21．10．05

受理日：平成21．11．09

特発性肺ヘモジデローシスを合併した新生児期発症血球貪食性リンパ組織球症の1例

A Newborn Case of Hemophagocytic Lymphohistiocytosis with Idiopathic Pulmonary Hemosiderosis

神戸大学大学院　医学研究科内科系学講座　小児科学分野

Department of Pediatrics, Kobe University Graduate School of Medicine

藤岡一路・森岡一朗・三輪明弘・齋藤敦郎・橋本総子・森川　悟・柴田暁男・横山直樹・松尾雅文

Kazumichi FUJIOKA，Ichiro MORIOKA，Akihiro MIWA，Atsuro SAITO，Fusako HASHIMOTO，Satoru MORIKAWA，Akio SHIBATA，Naoki YOKOYAMA，Masafumi MATSUO

Key Words：hemophagocytic lymphohistiocytosis（HLH），pulmonary hemorrhage，idiopathic pulmonary hemosiderosis （IPH），newborn

　血球貪食性リンパ組織球症（hemophagocytic lymphohistiocytosis：HLH）と特発性肺ヘモジデローシス（idiopathic pulmonary hemosiderosis：IPH）は，ともにマクロファージの活性化がその病態の発症に関与している。しかし，いまだHLHとIPHが合併した症例の報告はない。我々は，HLHとIPHを合併した新生児の1例を経験したので報告する。症例は，在胎37週4日，出生体重2,758gの男児。日齢15に肺出血にて発症。血液検査にて，低フィブリノゲン血症を含む凝固能異常の所見に加え，フェリチンが著増していた。その後，発熱，血小板減少が出現し，NK細胞活性の欠損，骨髄での血球貪食像を確認し，HLHと診断した。集中治療により凝固能異常が改善後も他部位に出血症状がないにもかかわらず肺出血を繰り返した。そこで，気管分泌液を解析したところ，ベルリン青染色陽性の組織球を確認し，IPHと診断した。本症例はHLHとIPHの合併を確認した初めての報告である。

　The pathogenesis of both hemophagocytic lymphohistiocytosis（HLH）and idiopathic pulmonary hemosiderosis（IPH） is closely related with activated macrophages. However, no cases of HLH with IPH have been reported. Our present case is a male newborn who was born at 37-weeks gestation with a birth weight of 2,758g. Respiratory and circulation managements including inhaled nitric oxide were required for his persistent pulmonary hypertension of the newborn during 1-week after birth. At 15 days after birth, pulmonary hemorrhage was occurred, and then he had clinical symptoms and laboratory findings, such as fever, coagulation disorders including low blood level of fibrinogen, thrombocytopenia, a low NK cell activity, high serum level of ferritin and soluble interleukin-2 receptor, and hemophagocytosis in bone marrow, which satisfy the criteria of HLH. Although his coagulation disorder was almost improved by intensive cares, his pulmonary hemorrhage was repeated. As recurrent pulmonary hemorrhage was not a major symptom of HLH, a cytological test in his bronchoalveolar lavage fluid was performed. It revealed that a histiocyte had some berlin blue positive hemosiderins, which means the diagnosis of IPH. Dexamethasone, cyclosporine A, dexamethasone palmitate, and methylpredonisolone pulse were used as treatments of HLH. However, he was not saved. To the best of our knowledge, this is the first case of HLH with IPH.