Study on the comprehensive treatment strategy of hemophagocytic syndrome

2014 Fiscal Year Final Research Report

• Project/Area Number: 24591577
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Pediatrics
• Research Institution: Yamaguchi University (2013-2014); Kyushu University (2012)
• Principal Investigator: OHGA Shouichi 山口大学, 医学(系)研究科(研究院), 教授 (60233053)
• Co-Investigator(Kenkyū-buntansha): TANAKA Tamami 九州大学, 医学研究院, 学術研究員 (60423547)
• Co-Investigator(Renkei-kenkyūsha): IMADOME Ken-ichi 国立成育医療センター研究所, 母児感染研究部・感染防御研究室, 室長 (70392488) ; OHSHIMA Koichi 久留米大学医学部, 第2病理学教室, 教授 (50203766)
• Project Period (FY): 2012-04-01 – 2015-03-31
• Keywords: 血球貪食症候群 / ＥＢウイルス / リンパ増殖性疾患 / 造血細胞移植 / 免疫化学療法 / 抗サイトカイン療法

Outline of Final Research Achievements

We studied the comprehensive treatment strategy of inherited or secondary hemophagocytic syndrome (hemophagocytic lymphohistiocytosis: HLH). In patients with inherited HLH, the utility and critical issues of unrelated donor umbilical cord blood transplantation (UCBT) as the curative therapy were reported. On the other hand, the early non-cytotoxic immunomodulation therapy was verified to be curable for patients with severe secondary HLH including Epstein-Barr virus (EBV)- associated HLH and neonatal herpes simplex virus (HSV)-associated HLH. Targeted cells of infection should be determined to exclude the underlying primary immunodeficiency diseases especially for the curative treatment of EBV-associated lymphoproliferative disease.

Free Research Field

医歯薬学