Recent Advances in the Diagnosis and Treatment of Hemophagocytic Lymphohistiocytosis: Clinical Approach to Cases in Japan

DOI 1 Citations 50 References
  • OHGA Shouichi
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University
  • ISHII Eiichi
    Department of Pediatrics, Faculty of Medicine, Saga University
  • TAKADA Hidetoshi
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University
  • NOMURA Akihiko
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University
  • HARA Toshiro
    Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University

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Other Title
  • 血球貪食性リンパ組織球症の病態解析と治療の進歩―症例へのアプローチ―
  • Clinical Approach to Cases in Japan
  • 症例へのアプローチ

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Hemophagocytic lymphohistiocytosis (HLH) is an immunohematologic emergency of infancy, which is characterized by high fever, hepatosplenomegaly, disseminated intravascular coagulopathy and bone marrow hemophagocytosis. This condition accounts for hypercytokinemia originating from the uncontrollable activation of lymphocytes and histiocytes associated with precipitating factors such as viral infection. The discovery of a perforin defect in familial HLH (FHL) patients led to the identification of responsible genes for inherited/primary HLH. Based on clinical data and experimental models, the molecular pathogenesis of primary HLH has been revealed to result from the defective metabolism of cytotoxic granules, by which cytotoxic T-cell processes govern the immune homeostasis. Primary immunodeficiency diseases and other genetic diseases are also associated with HLH, although the pathogenesis remains elusive. On the other hand, the genetic backgrounds of acquired/secondary HLH, including virus-or autoimmunity-associated HLH, have not been specified. The heterogeneity of underlying conditions and the diversity of treatment choices of HLH make it difficult for pediatricians to manage patients. Since the Epstein-Barr virus-HLH is occasionally encountered in Japan, foreign-made diagnostic guidelines and treatment protocols of FHL should be carefully applied to Japanese patients. We review recent advances in the diagnosis and management of HLH, and discuss the clinical approach to cases in Japan.

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