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Allogeneic Hematopoietic Cell Transplantation Ameliorated Asymptomatic Granulomatous and Lymphocytic Interstitial Lung Disease in a Patient With XIAP Deficiency
被引:0
|作者:
Oshima, Mai
[1
]
Matsukawa, Yukihiro
[1
,2
]
Ikeda, Yuhachi
[1
]
Sakamoto, Kenichi
[1
]
Taga, Takashi
[1
]
Maruo, Yoshihiro
[1
]
机构:
[1] Shiga Univ Med Sci, Dept Pediat, Otsu, Japan
[2] Shiga Univ Med Sci, Dept Pediat, Seta Tsukinowa Cho, Otsu, Shiga 5202192, Japan
关键词:
X-linked inhibitor of apoptosis protein deficiency;
granulomatous and lymphocytic interstitial lung disease;
hematopoietic cell transplantation;
D O I:
10.1097/MPH.0000000000002819
中图分类号:
R73 [肿瘤学];
学科分类号:
100214 ;
摘要:
X-linked inhibitor of apoptosis protein (XIAP) deficiency is an inborn error of immunity (IEI). Allogeneic hematopoietic cell transplantation (HCT) is currently the only curative therapy available for XIAP deficiency. Granulomatous and lymphocytic interstitial lung disease (GLILD) is a common immune-related lung complication of IEIs. We present a 6-year-old boy with XIAP deficiency and GLILD. Computed tomography showed lung nodes but no symptoms. Before HCT, GLILD was not managed with immunosuppressive therapy, because he was asymptomatic. The HCT procedure was subsequently performed. The post-HCT course was uneventful; follow-up computed tomography on day 46 showed nodules had disappeared. HCT could potentially ameliorate GLILD like other inflammatory processes associated with the underlying IEIs.
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页码:e191 / e194
页数:4
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