Two cases report of heavy- and light-amyloidosis and literature review
10.3760/cma.j.cn441217-20191219-00084
- VernacularTitle:重+轻链淀粉样变性二例报道并文献复习
- Author:
Jingyi BI
1
;
Bao DONG
;
Lei WEN
;
Wenbing DUAN
;
Xiaojun HUANG
;
Jin LU
Author Information
1. 北京大学人民医院血液科，北京大学血液病研究所，国家血液系统疾病临床医学研究中心，北京 100044
- From:
Chinese Journal of Nephrology
2020;36(6):447-453
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical manifestation, diagnosis and prognosis of heavy- and light-amyloidosis (AHL).Methods:The clinical data of two patients with AHL in Peking University People's Hospital and 21 cases of reported literature were reviewed to clarify the clinical and prognostic characteristics of AHL.Results:Compared with light-amyloidosis, most AHL patients were male, with high positive rate of blood and urine immunofixation electrophoresis and complete immunoglobulin. The manifestations of the kidneys were proteinuria, mainly composed of albumin, nephrotic syndrome and microscopic haematuria. The pathology of renal biopsy showed that Congo red staining positive substances were deposited in many sites (mesangial area, capillary wall, arteriole and renal interstitium), and immunofluorescence showed that monoclonal heavy chain with light chain (the type was consistent with the hematuria M protein) were clumpy deposition, which was consistent with amyloid deposition site. Heart involvement was rare, and the proportion of plasma cells in bone marrow was high.Conclusion:AHL is rare and its clinical manifestations of AHL are different from those of light-amyloidosis. The prognosis needs to be further observed.
