The spectrum of thrombotic microangiopathy related to monoclonal gammopathy.

ABSTRACT:

Background

Recent studies showed a high prevalence of monoclonal gammopathy (MG) in patients with thrombotic microangiopathy (TMA) aged over 50 years and suggested that complement dysregulation is pivotal for the disease to develop. Here, we studied this premise in seven patients with TMA and coexisting MG.

Methods

Patients with TMA on kidney biopsy and/or peripheral blood were recruited from the prospective COMPETE cohort (NCT04745195) and Limburg Renal Registry. Patients were screened for complement dysregulation, including genetics/factor H autoantibodies (FHAA) and functional ex vivo testing on microvascular endothelial cells.

Results

Seven (8%) out of 84 patients with TMA presented with a coexisting MG. MG clustered in patients aged over 50 years (n/N <

SUBMITTER: van Doorn DPC 

PROVIDER: S-EPMC10797488 | biostudies-literature | 2024 Jan

REPOSITORIES: biostudies-literature