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Splenectomy as an effective treatment for macrothrombocytopenia in Takenouchi-Kosaki syndrome
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  • Shio Yamano,
  • Akihiro Iguchi ,
  • Kotaro Ishikawa,
  • Atsushi Sakamoto,
  • Toru Uchiyama,
  • Kumiko Yanagi,
  • Tadashi Kaname,
  • Shinji Kunishima,
  • Akira Ishiguro
Shio Yamano
National Center for Child Health and Development

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Akihiro Iguchi
National Center for Child Health and Development
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Kotaro Ishikawa
National Center for Child Health and Development
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Atsushi Sakamoto
National Center for Child Health and Development
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Toru Uchiyama
National Research Institute for Child Health and Development
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Kumiko Yanagi
National Center for Child Health and Development
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Tadashi Kaname
National Center for Child Health and Development
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Shinji Kunishima
Gifu University of Medical Science
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Akira Ishiguro
National Center for Child Health and Development
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Abstract

Takenouchi-Kosaki syndrome (TKS) exhibits clinical features represented by macrothrombocytopenia, developmental delay, dysmorphic facial features, and deafness. There is little information on the treatment for macrothrombocytopenia in TKS. Splenectomy has been a contraindication for inherited thrombocytopenia. In the case of autoimmune haemolytic anaemia (AIHA) with TKS that we previously reported, AIHA initially resolved with prednisolone; however, it gradually became resistant to drug therapy. We performed splenectomy, and post-operatively, both anaemia and macrothrombocytopenia improved. This is a novel effect of splenectomy for thrombocytopenia in TKS, which suggests that splenectomy could be a treatment option for thrombocytopenia in TKS.
02 Dec 2022Published in International Journal of Hematology. 10.1007/s12185-022-03491-w