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Fever and Skin Eruption

  • Hirokazu KaneganeEmail author
Chapter

Abstract

  • A boy who presented with fever and skin eruptions was diagnosed as having type 1 X-Linked lymphoproliferative disease (XLP1)

  • Patients with EBV-hemophagocytic lymphohistiocytosis (HLH) refractory to conventional therapy and dysgammaglobulinemia are highly suspicious for XLP1

  • Patients with XLP1 show a complete lack of peripheral iNKT cells as well as patients with ITK, Coronin1A, and CTPS1 deficiencies

  • Rituximab and immunochemotherapy may be successful for the treatment of XLP1-associated EBV-HLH

Keywords

X-Linked lymphoproliferative disease Hemophagocytic lymphohistiocytosis EBV 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Child Health and DevelopmentGraduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU)TokyoJapan

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