Long-term CT findings of X-linked agammaglobulinemia with bronchiectasis diagnosed in an adult

Takashi Ishiguro, Noboru Takayanagi, Ichiro Kawada, Kazuyoshi Kurashima, Aya Matsushita, Keiji Harasawa, Noriko Tsuchiya, Koichiro Yoneda, Yousuke Miyahara, Shozaburo Yamaguchi, Ryozo Yano, Daido Tokunaga, Hiroo Saito, Mikio Ubukata, Tsutomu Yanagisawa, Yutaka Sugita

研究成果: Article査読


We report a case of X-linked agammaglobulinemia who presented with bronchiectasis. The patient had suffered pneumonia about every five years since childhood until he presented to our hospital at age 34 years old. CT showed bronchiectasis predominantly in the right middle lobe, lingula, and lower lobes. Administration of antibiotics resulted in symptomatic relief. Episodes of recurrent pulmonary infection and bronchiectasis indicated congenital immunodeficiency disorder. Investigation of lymphocyte subsets and serum immunoglobulin values showed remarkable reduction of B cells, IgG 772 mg/dl, IgA 216 mg/dl, and IgM 29 mg/dl. Flow cytometric assessment combined with genetic analysis was performed, and the results showed decreased expression of monocyte Bruton's tyrosine kinase (BTK) and missense mutation of Btk gene. We diagnosed X-linked agammaglobulinemia. IgG remained above 600 mg/dl in this case, we have not administered immunoglobulin after discharge. He suffered from pneumonia in 2004 and 2006 and bronchiectasis has progressed. In this report, we present a case including CT findings over a period of 8 years.

ジャーナルNihon Kokyūki Gakkai zasshi = the journal of the Japanese Respiratory Society
出版ステータスPublished - 2008 4

ASJC Scopus subject areas

  • Medicine(all)

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