Kosacka Monika, Wojtala Renata, Piesiak Paweł, Passowicz-Muszyńska Ewa, Jankowska Renata
Akademia Medyczna we Wrocławiu, Katedra i Klinika Pulmonologii i Nowotworbw Płuc.
Pol Merkur Lekarski. 2008 Dec;25(150):516-8.
Amyloidosis is a generic term for a heterogeneous group of disorders associated with deposition of protein in an abnormal fibrillar form. We report a case of kappa L-chain primary lung amyloidosis. A 44-year-old woman without symptoms. Roentgenogram and computed tomography of the chest showed several small nodular shadows. The mammography revealed tumor of the breast and the metastatic breast cancer have been suspected. After the quadrantectomy the breast tumor has been diagnosed as adenoma. The diagnosis of pulmonary amyloidosis was established by an open lung biopsy. Immunohistochemistry revealed amyloid light (AL) of kappa-light chain origin. Serum protein electrophoresis was negative for monoclonal gammapathy. Over the next 6 years, the patient remained relatively asymptomatic. The case illustrates the problems with diagnosis of multiple solitary nodules and the long and relatively benign course of primary pulmonary amyloidosis.
淀粉样变性是一组异质性疾病的通用术语，这些疾病与异常纤维状形式的蛋白质沉积有关。我们报告一例κ轻链原发性肺淀粉样变性病例。一名44岁无症状女性。胸部X线和计算机断层扫描显示有几个小结节阴影。乳房X线摄影显示乳房有肿瘤，怀疑是转移性乳腺癌。象限切除术后，乳房肿瘤被诊断为腺瘤。通过开胸肺活检确诊为肺淀粉样变性。免疫组织化学显示为κ轻链起源的淀粉样轻链（AL）。血清蛋白电泳对单克隆丙种球蛋白病呈阴性。在接下来的6年里，患者相对无症状。该病例说明了多发性孤立结节诊断中的问题以及原发性肺淀粉样变性的漫长且相对良性的病程。
