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与肿瘤病理学相关的免疫缺陷:古德综合征。

Immunodeficiency associated with tumour pathology: Good's syndrome.

作者信息

Barrios Recio Javier, Perez Rodriguez Alejandra, Callero Ariel, Martinez Tadeo Juan Antonio

机构信息

Department of Allergy, Hospital Universitario Nuestra Senora de la Candelaria, Santa Cruz de Tenerife, Spain.

Hospital Universitario Nuestra Senora de la Candelaria, Santa Cruz de Tenerife, Spain.

出版信息

BMJ Case Rep. 2019 Mar 20;12(3):e227970. doi: 10.1136/bcr-2018-227970.

Abstract

The Good's syndrome (GS) is a low prevalence entity where thymoma often is associated with immunodeficiency. Patients may start presenting recurrent rhinosinusal infections, bronchopulmonary infections, haematological alterations and diarrhoea, secondary to immunodeficiency. They can also present respiratory symptoms and parathymic syndromes derived from the existence of thymoma, a slow-growing neoplasm located in the anterior mediastinum. We present the case of a 76-year-old man diagnosed with thymoma by image analysis, which had presented multiple episodes of pneumonia and two admissions to the hospital for diarrhoea of weeks of evolution. After finishing the study, the patient is diagnosed of GS. In this case, thymectomy prevented the appearance of parathymic syndrome, but without any effect on immunodeficiency symptoms. To decrease repeat infections, substitution therapy with immunoglobulins was started. The prognosis will depend mainly on the recurrent infectious and to a lesser extent on the thymic neoplasm.

摘要

古德综合征(GS)是一种发病率较低的疾病,常伴有胸腺瘤和免疫缺陷。患者可能因免疫缺陷而开始出现反复的鼻-鼻窦炎感染、支气管肺部感染、血液学改变和腹泻。他们还可能出现因胸腺瘤(一种位于前纵隔的生长缓慢的肿瘤)存在而导致的呼吸道症状和胸腺瘤旁综合征。我们报告一例76岁男性患者,经影像学分析诊断为胸腺瘤,该患者曾多次发生肺炎,并因持续数周的腹泻两次入院。检查结束后,患者被诊断为GS。在本病例中,胸腺切除术预防了胸腺瘤旁综合征的出现,但对免疫缺陷症状没有任何影响。为减少反复感染,开始了免疫球蛋白替代治疗。预后主要取决于反复感染,其次取决于胸腺肿瘤。

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