Clinical conditions and risk factors for inhibitor-development in patients with haemophilia: A decade-long prospective cohort study in Japan, J-HIS2 (Japan Hemophilia Inhibitor Study 2).

Autor: Nogami K; Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan., Taki M; Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Kanagawa, Japan., Matsushita T; Department of Transfusion Medicine, Nagoya University Hospital, Nagoya, Japan., Kojima T; Aichi Health Promotion Foundation, Nagoya, Aichi, Japan.; Nagoya University, Nagoya, Aichi, Japan., Oka T; Department of Pediatrics, Sapporo Tokushukai Hospital, Sapporo, Japan., Ohga S; Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan., Kawakami K; Department of Pediatrics, Kagoshima City Hospital, Kagoshima, Japan., Sakai M; Department of Pediatrics, University of Occupational and Environmental Health Japan, Kitakyushu, Japan.; Department of Pediatrics, Munakata Suikokai General Hospital, Fukuoka, Japan., Suzuki T; Department of Blood Coagulation, Ogikubo Hospital, Tokyo, Japan., Higasa S; Department of Hematology, Hyogo College of Medicine, Nishinomiya, Japan., Horikoshi Y; Division of Hematology and Oncology, Shizuoka Children's Hospital, Shizuoka, Japan., Shinozawa K; Department of Laboratory Medicine, Tokyo Medical University, Tokyo, Japan., Tamura S; Division of Cellular and Genetic Sciences, Department of Integrated Health Sciences, Nagoya University Graduate School of medicine, Nagoya, Japan., Yada K; Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.; Division of Hemophilia, National Hospital Organization Osaka National Hospital, Osaka, Japan., Imaizumi M; Department of Hematology and Oncology, Miyagi Children's Hospital, Sendai, Japan., Ohtsuka Y; Department of Pediatrics, Hyogo College of Medicine, Nishinomiya, Japan., Iwasaki F; Division of Hematology and Oncology, Kanagawa Children's Medical Center, Yokohama, Japan., Kobayashi M; Department of Pediatrics, Hiroshima University Hospital, Hiroshima, Japan., Takamatsu J; Department of Transfusion Medicine, Nagoya University Hospital, Nagoya, Japan.; Japanese Red Cross Tokai Hokuriku Block Blood Center, Seto, Japan., Takedani H; Department of Joint Surgery, IMSUT hospital, The University of Tokyo, Tokyo, Japan., Nakadate H; Division of Hematology, Children's Cancer Center, National Center for Child Health and Development, Tokyo, Japan., Matsuo Y; Department of Pediatrics and Child Health, Kurume University School of Medicine, Kurume, Japan., Matsumoto T; Department of Transfusion Medicine and Cell Therapy, Mie University Hospital, Tsu, Japan., Fujii T; Division of Transfusion Medicine, Hiroshima University Hospital, Hiroshima, Japan., Fukutake K; Department of Blood Coagulation, Ogikubo Hospital, Tokyo, Japan.; Department of Laboratory Medicine, Tokyo Medical University, Tokyo, Japan., Shirahata A; Kitakyushu Yahata Higashi Hospital, Kitakyushu, Japan., Yoshioka A; Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan., Shima M; Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.
Jazyk: angličtina
Zdroj: Haemophilia : the official journal of the World Federation of Hemophilia [Haemophilia] 2022 Sep; Vol. 28 (5), pp. 745-759. Date of Electronic Publication: 2022 Jun 11.
DOI: 10.1111/hae.14602
Abstrakt: Background: Inhibitor-development is a serious complication in patients with haemophilia (PwH). Previous studies reported that therapeutic and genetic factors could be associated with these alloantibodies. Relevant clinical features such as genetic-background and different treatment regimens in Japan remain unclear, however.
Aims: To analyse a nation-wide Japanese registry for PwH, and to examine risk factors for inhibitor-development.
Methods and Results: Newly diagnosed patients with haemophilia A (PwHA) or haemophilia B (PwHB) without inhibitors after 2007, and with treatment records traceable from 0 to 75 exposure days (ED), were enrolled in the Japan Hemophilia Inhibitor Study 2 (J-HIS2) initiated in 2008. Of 417 patients (340 PwHA, 77 PwHB) from 46 facilities, 83 (76 PwHA, 7 PwHB) were recorded with inhibitors by July 2020. Inhibitors were observed in 31.0% of severe PwHA, 8.0% moderate and 1.6% mild and in 17.1% of severe PwHB. The majority of inhibitors (89.7% in severe PwHA and 71.4% in severe PwHB) were detected on or before 25ED (median 12ED in PwHA and 19ED in PwHB). Genotyping in these severe patients identified an association between inhibitor-development and null variants of F8 (P < .01) or F9 (P < .05). A lower incidence of inhibitors was recorded in severe PwHA treated with prophylaxis than in those treated on-demand (P < .01). A past-history of intracranial-haemorrhage appeared to be associated with inhibitor-development, while FVIII-concentrates infusion and routine vaccination on the same day was not related to inhibitor-development.
Conclusion: The J-HIS2 study has identified significant clinical variables associated with inhibitor-development in Japanese PwH, consistent with other global studies.
(© 2022 John Wiley & Sons Ltd.)
Databáze: MEDLINE
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