Autonomic nervous symptoms as the initial presentation in systemic light-chain amyloidosis with multiple myeloma: a case report
10.3760/cma.j.cn113694-20211015-00715
- VernacularTitle:以自主神经症状为首发表现的系统性轻链淀粉样变性合并多发性骨髓瘤1例
- Author:
Jie ZHANG
1
;
Xinrui LI
;
Weihao XING
;
Jing HE
;
Jing XIONG
Author Information
1. 昆明医科大学第二附属医院神经内科，昆明 650031
- Keywords:
Hypotension, orthostatic;
Autonomic nervous system;
Amyloidosis;
Multiple myeloma
- From:
Chinese Journal of Neurology
2022;55(7):743-746
- CountryChina
- Language:Chinese
-
Abstract:
Immunoglobulin light chain amyloidosis (AL) is characterized by the extracellular tissue deposition of insoluble fibrils as a result of protein misfolding. These tissue deposits may be responsible for progressive failure in several organs. Among them, neuropathy may be presented as the first manifestation. The patient reported here presented initially with autonomic nervous system impairment, mainly characterized by severe refractory orthostatic hypotension, which became progressively invalidating, forcing the patient to bed. Moreover, since the systemic involvement of the disease, the patient also presented with diarrhea, peripheral polyneuropathy, and kidney dysfunction. Eventually, the massive myocardial depression and infiltration led to a fatal outcome due to ventricular fibrillation. Examination revealed M protein in serum and urine protein electrophoresis. Rectal mucosa and skin biopsy confirmed amyloidosis, and bone marrow biopsy showed cellular infiltration was over 35% with 23% immature plasma cells. The patient was confirmed as AL with multiple myeloma.
