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Successful hematopoietic cell transplantation in a patient with X-linked chronic granulomatous disease concomitant with McLeod phenotype
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  • Kentaro Fujimori,
  • Soichiro Shimizu,
  • Takashi Ishikawa,
  • Yoshihiro Gocho,
  • Hirotoshi Sakaguchi,
  • Toru Uchiyama,
  • Kazuhiko Nakabayashi,
  • Daisuke Tomizawa,
  • Masafumi Onodera,
  • Kimikazu Matsumoto,
  • Toshinao Kawai,
  • Akihiro Iguchi
Kentaro Fujimori
Kokuritsu Kenkyu Kaihatsu Hojin Kokuritsu Seiiku Iryo Kenkyu Center Byoin
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Soichiro Shimizu
Kokuritsu Kenkyu Kaihatsu Hojin Kokuritsu Seiiku Iryo Kenkyu Center Byoin
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Takashi Ishikawa
Kokuritsu Kenkyu Kaihatsu Hojin Kokuritsu Seiiku Iryo Kenkyu Center Byoin
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Yoshihiro Gocho
Kokuritsu Kenkyu Kaihatsu Hojin Kokuritsu Seiiku Iryo Kenkyu Center Byoin
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Hirotoshi Sakaguchi
Kokuritsu Kenkyu Kaihatsu Hojin Kokuritsu Seiiku Iryo Kenkyu Center Byoin
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Toru Uchiyama
Kokuritsu Kenkyu Kaihatsu Hojin Kokuritsu Seiiku Iryo Kenkyu Center Byoin
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Kazuhiko Nakabayashi
Kokuritsu Kenkyu Kaihatsu Hojin Kokuritsu Seiiku Iryo Kenkyu Center Kenkyujo
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Daisuke Tomizawa
Kokuritsu Kenkyu Kaihatsu Hojin Kokuritsu Seiiku Iryo Kenkyu Center Byoin
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Masafumi Onodera
Kokuritsu Kenkyu Kaihatsu Hojin Kokuritsu Seiiku Iryo Kenkyu Center Byoin
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Kimikazu Matsumoto
Kokuritsu Kenkyu Kaihatsu Hojin Kokuritsu Seiiku Iryo Kenkyu Center Byoin
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Toshinao Kawai
Kokuritsu Kenkyu Kaihatsu Hojin Kokuritsu Seiiku Iryo Kenkyu Center Byoin
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Akihiro Iguchi
Kokuritsu Kenkyu Kaihatsu Hojin Kokuritsu Seiiku Iryo Kenkyu Center Byoin

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Abstract

X-linked chronic granulomatous disease (X-CGD) may be associated with McLeod syndrome (MLS) as a contiguous gene deletion syndrome. MLS is characterized by the loss of XK protein together with Kx antigen on red blood cell (RBC) surfaces and late-onset neurocognitive symptoms. X-CGD treatment involves hematopoietic cell transplantation (HCT), and severe hemolysis may occur due to Kx antigen sensitization. Here, we report successful HCT in a 7-year-old male with X-CGD and MLS achieved by depleting RBCs from the graft and adding rituximab to the conditioning regimen. No hemolytic events occurred, and he is doing well 2 years after HCT.