Article Text

Download PDFPDF
Long-term remission of cryopyrin-associated periodic syndrome after allogeneic haematopoietic stem cell transplantation
  1. Takehiko Mori1,
  2. Masuho Saburi2,
  3. Maki Hagihara3,
  4. Masaaki Mori4,
  5. Rie Yamazaki1,
  6. Jun Kato1
  1. 1 Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan
  2. 2 Department of Medical Oncology and Hematology, Oita University Faculty of Medicine, Oita, Japan
  3. 3 Department of Hematology and Clinical Immunology, Yokohama City University School of Medicine, Yokohama, Japan
  4. 4 Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
  1. Correspondence to Dr Takehiko Mori, Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan; tmori{at}

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Cryopyrin-associated periodic syndrome (CAPS) is an inherited inflammatory disorder.1 CAPS is characterised by skin rash, fever and the inflammations involving the eyes, ears, bones, joints and meninges. The mutations of NLRP3 encoding interleukin-1 (IL-1) inflammasome protein, cryopyrin, lead to the increased IL-1β secretion. The treatment for CAPS includes symptomatic treatments and IL-1β pathway blocking agents.2–4 Although such agents are effective, they are not curative. Allogeneic haematopoietic stem cell transplantation (HSCT) is widely performed for haematological disorders and some inherited immune disorders. Through allogeneic HSCT, the hosts’ haematopoietic and immune cells are completely replaced by the normal donor cells. Therefore, CAPS could be theoretically cured by allogeneic HSCT; however, there have been no reported patients with CAPS who underwent allogeneic HSCT so far.

A 30-year-old woman had a long-standing history of recurrent symptoms/signs such as fever, skin rash, arthralgia, serious headache and uveitis of the neonatal onset. She had presented a hearing loss since her …

View Full Text


  • Handling editor Josef S Smolen

  • Contributors TM, MS and JK treated the patients and wrote the manuscript; MH, MM diagnosed and treated the patient, and provided important opinions on the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests TM received research funding from MSD KK, Novartis Pharma, LSI Medience, Medical & Biological Laboratories, and Asahi Kasei Corporation, and personal fees from Pfizer, MSD, Janssen Pharma, Sumitomo Dainippon Pharma, Novartis Pharma, Kyowa Kirin, Chugai Pharmaceutical, Shionogi, Japan Blood Products Organization, Takeda Pharmaceutical, Ono Pharmaceutical, Shire, Eisai, and Astellas Pharma; MM received research funding from Chugai Pharmaceutical, UCB Japan, CSL Behring, Abbvie Japan, Japan Blood Products Organization, Ayumi Pharmaceutical, Nippon Kayaku and Asahi Kasei, personal fees from MSD KK, and consulting fees from Daiichi Sankyo, and Taisho Pharmaceutical.

  • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.