X-linked agammaglobulinemia (XLA) is a rare form of inherited immunodeficiency due to an impairment in B-lymphocyte differentiation and maturation. In the majority of cases XLA is diagnosed in childhood, particularly among males affected by recurrent infections and with a family history of immunodeficiency. Infections of respiratory tract, gastrointestinal apparatus, eyes, nose and ears are frequent in XLA patients; on the contrary, infections of myocardium, cardiac valves and pericardium are rarely described in XLA. A 34-year-old man with unknown XLA was hospitalized because of syncope, due to pericardial tamponade, caused by acute primary purulent pericarditis. Immediate pericardiocentesis was effective in improving hemodynamics, and empiric antibiotic therapy was successful in controlling the infection. Purulent pericarditis is a rare disease with high mortality rate: it is usually caused by hematogenous bacterial propagation, direct infection of pericardial space by chest wounds or thoracic surgery, or extension of infection from adjacent tissues. However, this patient had no recent local or systemic infections. Because of unusual clinical picture during hospitalization he underwent further clinical and laboratory evaluations, that showed low immunoglobulin levels. After exclusion of acquired immunodeficiency, genetic tests were performed: they detected deletion of exons 8-9-10 of Bruton Tyrosine Kinase gene on X chromosome, leading to the diagnosis of XLA. Acute purulent primary pericarditis may also occur in adult XLA patients as first clinical manifestation. According to this case report, a primary immunodeficiency syndrome should be considered in patients with atypical cardiac infections and no predisposing conditions, regardless of age.

中文翻译：

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患有未知X连锁无球蛋白血症的成年患者的急性原发性化脓性心包炎。

由于B淋巴细胞分化和成熟受到损害，X连锁无球蛋白血症（XLA）是一种罕见的遗传性免疫缺陷形式。在大多数情况下，XLA是在儿童时期被诊断出的，尤其是在反复感染且有免疫缺陷家族史的男性中。XLA患者常见呼吸道，胃肠道，眼睛，鼻子和耳朵的感染；相反，XLA很少描述心肌，心脏瓣膜和心包的感染。一名XLA未知的34岁男性因急性原发性化脓性心包炎引起的心包填塞引起晕厥而入院。立即的心包穿刺术可有效改善血液动力学，经验性抗生素治疗可成功控制感染。化脓性心包炎是一种罕见的高死亡率疾病：它通常是由血源性细菌繁殖，胸部伤口或胸腔外科手术直接感染心包腔或邻近组织的感染扩大所致。但是，该患者近期没有局部或全身感染。由于住院期间的异常临床情况，他接受了进一步的临床和实验室评估，结果显示免疫球蛋白水平较低。排除获得性免疫缺陷后，进行基因测试：他们检测到X染色体上Bruton酪氨酸激酶基因外显子8-9-10的缺失，从而诊断出XLA。成人XLA患者也可能发生急性化脓性原发性心包炎，这是其最初的临床表现。根据这份个案报告，