[Long-term CT findings of X-linked agammaglobulinemia with bronchiectasis diagnosed in an adult].
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.
Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center.
Takashi
T
Ishiguro
Noboru
N
Takayanagi
Ichiro
I
Kawada
Kazuyoshi
K
Kurashima
Aya
A
Matsushita
Keiji
K
Harasawa
Noriko
N
Tsuchiya
Koichiro
K
Yoneda
Yousuke
Y
Miyahara
Shozaburo
S
Yamaguchi
Ryozo
R
Yano
Daido
D
Tokunaga
Hiroo
H
Saito
Mikio
M
Ubukata
Tsutomu
T
Yanagisawa
Yutaka
Y
Sugita
D000328:Adult; D000361:Agammaglobulinemia / Q000000981:diagnostic imaging* / Q000235:genetics; D001987:Bronchiectasis / Q000000981:diagnostic imaging*; D040181:Genetic Diseases, X-Linked; D006801:Humans; D008297:Male; D014057:Tomography, X-Ray Computed