Work

Neurogenic orthostatic hypotension as a rare presentation of Waldenström macroglobulinemia associated with light-chain amyloidosis: A diagnostic challenge

Year: 2025

Type: article

Abstract: Neurogenic orthostatic hypotension (nOH) is frequently associated with synucleinopathies like pure autonomic failure (PAF). However, systemic diseases may mimic this presentation, posing a diagnostic challenge. We report a patient with isolated nOH initially suggestive of PAF. Detailed work-up revealed Waldenström macroglobulinemia with associated AL amyloidosis (WM-AL), affecting isolated postganglionic autonomic fibers but without classic systemic organ involvement. This case underscores the importance of considering secondary causes in patients with isolated autonomic dysfunction. Unlike idiopathic PAF, paraproteinemic neuropathies such as WM-AL require distinct diagnostic and therapeutic approaches. WM-AL can mimic primary autonomic disorders like PAF and should be included in the differential diagnosis of nOH, even in the absence of systemic manifestations.

Source: Journal of Neurosciences in Rural Practice

DOI: 10.25259/jnrp_163_2025

Authors: Jesus Alfonso Romero-Mera, Jose Antonio Aguilar-Hidalgo, Daniel Rebolledo Garcia

Institutions: NYU Langone Health, New York University, Institute for Social Security and Services for State Workers

Language: English

Topic: Cardiovascular Syncope and Autonomic Disorders

Subfield: Surgery

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $125