TY - JOUR
T1 - Severe neutropenia in Japanese patients with X-linked agammaglobulinemia
AU - Kanegane, Hirokazu
AU - Taneichi, Hiromichi
AU - Nomura, Keiko
AU - Futatani, Takeshi
AU - Miyawaki, Toshio
N1 - Funding Information:
We are grateful to all participating patients and their families for cooperation in this study. We also thank Mrs Chikako Sakai and Mr Hitoshi Moriuchi for their technical assistance. This study was supported by the Ministry of Education, Culture, Sports, Science and Technology, Japan, and the Ministry of Health, Labour and Welfare, Japan. We are also indebted to all physicians, especially the following, for their responses to the second questionnaires: Hyakuna N, Ryukyu University, Nishihara; Imanishi H, NTT West Osaka Hospital, Osaka; Ishihara S, Kashiwara City Hospital, Kashiwara; Murakami G, Toyama Red Cross Hospital, Toyama; Nakayama Y, Showa Ina General Hospital, Komagane; Oh-ishi T, Saitama Prefectural Children’s Medical Center, Iwatsuki; Ohsaki M, Hakodate Municipal Hospital, Hakodate; Sakazume S, Koshigaya Hospital, Dokkyo University School of Medicine, Koshigaya; Sako M, Osaka City General Hospital, Osaka; Shiota M, Kishiwada City Hospital, Kishiwada; Tamagami C, Chugoku Rosai Hospital, Kure; Wakiguchi H, Kochi Medical School, Nangoku; Yamaga H, Naga Hospital, Uchida; Yamamoto T, National Sengokuso Hospital, Kaizuka.
PY - 2005/9
Y1 - 2005/9
N2 - X-linked agammaglobulinemia (XLA) is clinically characterized by recurrent bacterial infections during early infancy. Although it is not a phagocytic disorder, XLA is sometimes associated with neutropenia. We conducted a nation-wide survey to determine the frequency of neutropenia among Japanese XLA patients. Responses were received from 87 (86%) of 101 patients in which BTK mutations were previously identified, and of these, 16 (18%) had neutropenia. All episodes of neutropenia occurred before initiation of intravenous immunoglobulin (IVIG) replacement therapy. Two XLA patients died of multiple organ failure caused by severe neutropenia and Pseudomonas sepsis before initiation of IVIG replacement therapy. These results suggest that, in some cases, severe bacterial infections in XLA patients might be caused not only by antibody deficiencies but also by neutropenia.
AB - X-linked agammaglobulinemia (XLA) is clinically characterized by recurrent bacterial infections during early infancy. Although it is not a phagocytic disorder, XLA is sometimes associated with neutropenia. We conducted a nation-wide survey to determine the frequency of neutropenia among Japanese XLA patients. Responses were received from 87 (86%) of 101 patients in which BTK mutations were previously identified, and of these, 16 (18%) had neutropenia. All episodes of neutropenia occurred before initiation of intravenous immunoglobulin (IVIG) replacement therapy. Two XLA patients died of multiple organ failure caused by severe neutropenia and Pseudomonas sepsis before initiation of IVIG replacement therapy. These results suggest that, in some cases, severe bacterial infections in XLA patients might be caused not only by antibody deficiencies but also by neutropenia.
KW - Bruton's tyrosine kinase (BTK)
KW - Intravenous immunoglobulin (IVIG)
KW - Neutropenia
KW - X-linked agammaglobulinemia (XLA)
UR - http://www.scopus.com/inward/record.url?scp=24744468059&partnerID=8YFLogxK
U2 - 10.1007/s10875-005-5370-x
DO - 10.1007/s10875-005-5370-x
M3 - 学術論文
C2 - 16160918
AN - SCOPUS:24744468059
SN - 0271-9142
VL - 25
SP - 491
EP - 495
JO - Journal of Clinical Immunology
JF - Journal of Clinical Immunology
IS - 5
ER -