Manoj Edirisooriya Maddumage, Srigrishna Rajini, Ragunathan Murugapillai K
Ward 45, National Hospital of Sri Lanka, Colombo, Sri Lanka.
J Med Case Rep. 2012 Jul 12;6:195. doi: 10.1186/1752-1947-6-195.
Isolated hepatic tuberculosis is an uncommon manifestation of one of the most common infections worldwide, caused by Mycobacterium tuberculosis. Extremely high serum ferritin, which is regarded as a marker of adult onset Still's disease, has not been observed in patients with tuberculosis of the liver. We report a case of hepatic tuberculosis who presented with clinical criteria of adult-onset Still's disease and extreme hyperferritinemia, which posed a diagnostic confusion.
Our patient was a 48-year-old Sri Lankan man who presented with fever, polyarthralgia and a generalized skin rash of three months duration. He had marked constitutional symptoms, oral ulcers, hair loss, anemia and hepatomegaly. Laboratory investigations disclosed an inflammatory syndrome, evidence of hepatic dysfunction, bone marrow suppression and a raised serum ferritin level of 34,674 ng/ml. A rapidly deteriorating course of illness prompted treatment based on a presumptive diagnosis of adult-onset Still's disease until liver histology was available. The patient died of sepsis followed by multi-organ dysfunction. Later, the liver histology revealed tuberculosis.
Extrapulmonary tuberculosis, although well known to present with peculiar manifestations, has not been reported to be associated with extremely high levels of serum ferritin in immunocompetent individuals. Isolated hepatic tuberculosis presenting with clinical criteria of adult-onset Still's disease is remarkable. Since tuberculosis remains a potentially curable disease, an awareness of its' protean manifestations is essential.
孤立性肝结核是全球最常见感染之一——结核分枝杆菌感染的一种罕见表现形式。在肝结核患者中尚未观察到被视为成人斯蒂尔病标志物的极高血清铁蛋白水平。我们报告一例肝结核病例,该病例呈现出成人斯蒂尔病的临床标准及极高的铁蛋白血症,这造成了诊断上的困惑。
我们的患者是一名48岁的斯里兰卡男性,出现发热、多关节痛及持续3个月的全身性皮疹。他有明显的全身症状、口腔溃疡、脱发、贫血及肝肿大。实验室检查显示有炎症综合征、肝功能障碍证据、骨髓抑制以及血清铁蛋白水平升高至34674 ng/ml。病情迅速恶化促使在获得肝脏组织学结果之前,根据成人斯蒂尔病的推测性诊断进行治疗。患者死于败血症,随后出现多器官功能障碍。后来,肝脏组织学检查显示为结核。
肺外结核虽然以特殊表现而闻名,但在免疫功能正常的个体中尚未报道与极高的血清铁蛋白水平相关。以成人斯蒂尔病临床标准表现的孤立性肝结核很是罕见。由于结核病仍然是一种潜在可治愈的疾病,认识到其多样的表现形式至关重要。