Division of Hematology/Oncology, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung, Taiwan.
Am J Med Sci. 2012 Jul;344(1):69-71. doi: 10.1097/MAJ.0b013e31824a8927.
Franklin disease, or gamma heavy-chain disease, in patients with autoimmune disorders is a challenge for clinicians to diagnose due to its rarity, and recurrent infection is one of its characteristics. Within the spectrum of infections in Franklin disease patients, various fungi should always be considered. In this study, the authors describe a 57-year-old non-human immunodeficiency virus-infected systemic lupus erythematosus patient later diagnosed with Franklin disease and then developed Penicillium pneumonia. Because of the unexpected combination of Franklin disease and Penicillium infection in a non-human immunodeficiency virus-infected patient, the diagnosis of common hospital-acquired pneumonia was initially made. The laboratory examinations and cultures helped confirm the correct diagnosis of Franklin disease and Penicillium pneumonia. This is the first report of Penicillium sp. infection in a patient with Franklin disease, and it emphasizes the importance of proper preparation for biopsy, complete hematologic investigation, culture preparation and early antifungal coverage to improve the outcome.
弗兰克林病,又称γ重链病,在自身免疫性疾病患者中较为罕见,给临床医生的诊断带来挑战,其特征之一是反复感染。在弗兰克林病患者的感染谱中,各种真菌都应始终被考虑在内。在本研究中,作者描述了一位 57 岁的非人类免疫缺陷病毒感染的系统性红斑狼疮患者,后被诊断为弗兰克林病,继而又发生了青霉病。由于在非人类免疫缺陷病毒感染患者中出现了弗兰克林病和青霉病的意外组合,最初诊断为常见的医院获得性肺炎。实验室检查和培养有助于确认弗兰克林病和青霉病的正确诊断。这是首例弗兰克林病合并青霉属感染的病例报告,强调了适当准备活检、全面血液学检查、培养准备和早期抗真菌覆盖以改善预后的重要性。