title: Hemophagocytic lymphohistiocytosis with leukoencephalopathy in a patient with dermatomyositis accompanied with peripheral T-cell lymphoma: a case report.
abstract: BACKGROUND
  Hemophagocytic lymphohistiocytosis associated with autoimmune diseases is seen in patients with systemic juvenile idiopathic arthritis, adult-onset Still's disease, and systemic lupus erythematosus, whereas it is rarely seen in patients with dermatomyositis. In addition, central nervous system involvement with dermatomyositis is rare. To the best of our knowledge, this is the first case of hemophagocytic lymphohistiocytosis complicated by leukoencephalopathy in a patient with dermatomyositis accompanied with peripheral T-cell lymphoma.
  
  
  METHODS
  A 17-year-old Asian male adolescent with dermatomyositis and hemophagocytic lymphohistiocytosis that were controlled with corticosteroid therapy presented to our hospital with high fever and altered consciousness. Brain magnetic resonance imaging revealed multiple cerebral lesions. We diagnosed the central nervous system lesions as leukoencephalopathy secondary to dermatomyositis and hemophagocytic lymphohistiocytosis. Because corticosteroid and cyclophosphamide pulse therapy was ineffective, he was treated with a modified hemophagocytic lymphohistiocytosis-2004 protocol, which resulted in the disappearance of the lesions of his central nervous system.
  
  
  CONCLUSIONS
  Our findings suggest that the hemophagocytic lymphohistiocytosis-2004 protocol including etoposide should be initiated immediately in patients with hemophagocytic lymphohistiocytosis who respond poorly to treatment for the underlying disease. Moreover, irrespective of the underlying disease, patients with hemophagocytic lymphohistiocytosis with central nervous system lesions might require bone marrow transplantation.
authors.affiliation: Department of Rheumatology and Allergology, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano, Osaka, 586-8521, Japan.
authors.forename: Satoru
authors.initials: S
authors.lastname: Teshigawara
authors.affiliation: Department of Rheumatology and Allergology, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano, Osaka, 586-8521, Japan.|Department of General Internal Medicine, Graduate Medical Education and Clinical Investigation, Sakai City Medical Center, Ebaraji-cho 1-1-1, West Ward, Sakai, Osaka, 593-8304, Japan.
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Katada
authors.affiliation: Department of Rheumatology and Allergology, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano, Osaka, 586-8521, Japan.
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Maeda
authors.affiliation: Department of Rheumatology and Allergology, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano, Osaka, 586-8521, Japan.
authors.forename: Maiko
authors.initials: M
authors.lastname: Yoshimura
authors.affiliation: Department of Rheumatology and Allergology, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano, Osaka, 586-8521, Japan.
authors.forename: Eriko
authors.initials: E
authors.lastname: Kudo-Tanaka
authors.affiliation: Department of Rheumatology and Allergology, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano, Osaka, 586-8521, Japan.
authors.forename: Soichiro
authors.initials: S
authors.lastname: Tsuji
authors.affiliation: Department of Rheumatology and Allergology, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano, Osaka, 586-8521, Japan.
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Harada
authors.affiliation: Department of Rheumatology and Allergology, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano, Osaka, 586-8521, Japan.
authors.forename: Masato
authors.initials: M
authors.lastname: Matsushita
authors.affiliation: Department of Clinical Research, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano, Osaka, 586-8521, Japan.
authors.forename: Shiro
authors.initials: S
authors.lastname: Ohshima
authors.affiliation: Department of Stroke Center, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano, Osaka, 586-8521, Japan.
authors.forename: Kotaro
authors.initials: K
authors.lastname: Watanabe
authors.affiliation: Division of Hematology and Rheumatology, Kinki University Faculty of Medicine, 377-2 Ono-higashi, Osaka-sayama, Osaka, 589-8511, Japan.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Kumode
authors.affiliation: Department of Pathology, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano, Osaka, 586-8521, Japan.
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Hoshida
authors.affiliation: Department of Clinical Research, NHO Osaka-Minami Medical Center, 2-1 Kidohigashi-machi, Kawachinagano, Osaka, 586-8521, Japan. saekiy@ommc-hp.jp.
authors.forename: Yukihiko
authors.initials: Y
authors.lastname: Saeki
mesh_terms: D000293:Adolescent; D001921:Brain / Q000000981:diagnostic imaging; D003882:Dermatomyositis / Q000150:complications* / Q000175:diagnosis; D003937:Diagnosis, Differential; D006801:Humans; D056784:Leukoencephalopathies / Q000150:complications* / Q000000981:diagnostic imaging; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications*; D016411:Lymphoma, T-Cell, Peripheral / Q000150:complications* / Q000000981:diagnostic imaging; D008279:Magnetic Resonance Imaging; D008297:Male
keywords: Dermatomyositis; HLH-2004 protocol; Hemophagocytic lymphohistiocytosis; Leukoencephalopathy; Peripheral T-cell lymphoma