Nguyen T U, Oghalai J S, McGregor D K, Janssen N M, Huston D P
Immunology Allergy and Rheumatology Section, Department of Medicine, Baylor College of Medicine, Houston, TX 77030-3498, USA.
Hum Pathol. 2001 Mar;32(3):346-8. doi: 10.1053/hupa.2001.22742.
Amyloidosis typically manifests with disseminated infiltration of multiple organ systems. Rarely, amyloidosis may be localized. We report a patient with localized subcutaneous nodular amyloidosis, without systemic amyloid involvement or myeloma, whose presenting symptom was multiple discrete neck nodules. Immunohistochemical analysis showed the amyloid deposits to be derived from lambda light chains. Twenty-four month follow-up showed minimal disease progression. A literature review showed only 5 reported cases of subcutaneous nodular amyloidosis. This is the first description of a patient with subcutaneous nodular amyloidosis derived from lambda light chains. HUM PATHOL 32:346-348.
淀粉样变性通常表现为多个器官系统的弥漫性浸润。淀粉样变性很少会局限存在。我们报告一例局限性皮下结节性淀粉样变性患者,无系统性淀粉样变累及或骨髓瘤,其首发症状为多个散在的颈部结节。免疫组化分析显示淀粉样沉积物源自λ轻链。24个月的随访显示疾病进展轻微。文献综述显示仅报道过5例皮下结节性淀粉样变性病例。这是首例关于源自λ轻链的皮下结节性淀粉样变性患者的描述。《人类病理学》32:346 - 348。