98%
921
2 minutes
20
Systemic light chain (AL) amyloidosis stems from abnormal production of amyloidogenic immunoglobulin light chains by plasma cells or B-cell disorders. It can present locally or systemically, with systemic forms posing significant mortality risks, especially if cardiac involvement is present. Delayed diagnosis due to nonspecific symptoms leads to progressive organ dysfunction. Early recognition is vital for timely treatment, aiming to suppress amyloid production and reduce organ damage, thus promoting recovery and improving survival rates. This review article provides a comprehensive overview of the recent advancements in medical therapy for patients with AL cardiac amyloidosis.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1016/j.hfc.2024.09.005 | DOI Listing |
Neurology
October 2025
Department of Neurology, Mayo Clinic, Rochester, MN.
Monoclonal gammopathy-associated myopathies (MGAMs) are rare yet treatable myopathies that occur in association with monoclonal gammopathies. These myopathies include light chain (AL) amyloidosis myopathy, sporadic late-onset nemaline myopathy (SLONM), scleromyxedema with associated myopathy, and newly reported monoclonal gammopathy-associated glycogen storage myopathy (MGGSM), including the vacuolar myopathy with monoclonal gammopathy and stiffness. All these 4 distinct subtypes of MGAMs typically present in patients aged 40 or older, frequently with a subacute onset of rapidly progressive proximal and axial muscle weakness.
View Article and Find Full Text PDFNeurology
October 2025
Alzheimer's Disease and Other Cognitive Disorders Unit, Department of Neurology, Hospital Clínic de Barcelona, Fundació Recerca Clínic Barcelona-IDIBAPS, Spain.
Background And Objectives: α-Synuclein seed amplification assays (αSAAs) can improve the diagnosis of synucleinopathies and detect α-synuclein (αSyn) copathology in vivo in clinical practice. We aimed to evaluate the diagnostic performance of αSAA for detecting αSyn in CSF for diagnosing dementia with Lewy bodies (DLB) in a clinical cohort of cognitively impaired individuals. We explored how the coexistence of Alzheimer disease (AD) and αSyn pathology influences biomarker levels and clinical profiles.
View Article and Find Full Text PDFBlood Adv
September 2025
Massachusetts General Hospital Cancer Center, Boston, Massachusetts, United States.
Mass spectrometry (MS) is an emerging tool in multiple myeloma that detects and quantifies monoclonal proteins in the peripheral blood with sensitivity several orders of magnitude greater than conventional serum protein electrophoresis and immunofixation. Both intact light chain (top-down) and clonotypic peptide (bottom-up) MS approaches have demonstrated sensitivity comparable to-or even surpassing-BM-based assessments using next generation flow cytometry or sequencing. However, due to the delayed clearance of paraproteins, MS may be less informative for early response assessment, underscoring the need to define the optimal timing for evaluation.
View Article and Find Full Text PDFCureus
August 2025
Neurological Surgery, Punjab Institute of Neurosciences, Lahore, PAK.
Parvovirus B19 (PVB19) is an infrequent, serious, yet treatable cause of infection in immunocompromised hosts. Neurological manifestations of PVB19 are encephalitis, encephalopathy, meningitis, cerebellar ataxia, transverse myelitis, stroke, and peripheral neuropathy. The objective is to identify the exact clinical and diagnostic features specific to parvovirus B19 encephalitis for the isolation and management of the pathology.
View Article and Find Full Text PDFFront Immunol
September 2025
Northwell, New Hyde Park, NY, United States.
Immunoglobulins (IGs) made by chronic lymphocytic leukemia (CLL) B cells are unique in that they bind themselves (homo-dimerize). This interaction leads to signal transduction with functional consequences that depend on the affinity of homo-dimerization. We have studied the antigen-binding properties of the IGs from a subset of patients with CLL (Subset #4) that homo-dimerize at high affinity.
View Article and Find Full Text PDF