Cicco Sebastiano, Solimando Antonio G, Leone Patrizia, Battaglia Stefano, Ria Roberto, Vacca Angelo, Racanelli Vito
Department of Biomedical Sciences and Human Oncology, Section of Internal Medicine "G. Baccelli", University of Bari Aldo Moro Medical School, Bari, Italy.
Case Rep Hematol. 2018 Oct 17;2018:8606430. doi: 10.1155/2018/8606430. eCollection 2018.
Primary systemic amyloidosis is not easily diagnosed. The immunoglobulin deposits are usually localized in the kidney, heart, and liver. We describe an unusual case of a patient suffering from a pericardial amyloidoma with internal calcifications and air bubbles that compressed the right ventricle and shifted the heart to the left. Since the patient was in shock, urgent pericardiotomy was performed. This site showed PET uptake. A monoclonal component was present. On these findings, differential diagnoses included multiple myeloma and atypical pericardial tuberculosis, whereas a periumbilical fat tissue biopsy demonstrated amyloidosis. A previous species infection had most likely stimulated the production of amyloid. The patient received bortezomib/dexamethasone treatment and achieved a good response.
原发性系统性淀粉样变性不易诊断。免疫球蛋白沉积物通常定位于肾脏、心脏和肝脏。我们描述了一例不寻常的病例，患者患有心包淀粉样瘤，伴有内部钙化和气泡，压迫右心室并使心脏向左移位。由于患者处于休克状态，紧急进行了心包切开术。该部位显示有PET摄取。存在单克隆成分。基于这些发现，鉴别诊断包括多发性骨髓瘤和非典型心包结核，而脐周脂肪组织活检显示为淀粉样变性。先前的某种感染很可能刺激了淀粉样蛋白的产生。患者接受硼替佐米/地塞米松治疗并取得了良好疗效。
