2026-08-13 | Amyloidosis and Thoracic Aortic Disease: A Scoping Review.
Background and Objectives: Amyloidosis is a systemic disorder characterized by extracellular deposition of misfolded protein fibrils, most commonly light-chain (AL) or transthyretin-derived (ATTR) ones. Cardiac involvement is well recognized, but large-vessel complications, including thoracic aortic aneurysms (TAA) and dissections, are rare and under-reported. The aim of this review article is to provide insights into pathophysiology, clinical diagnosis and the therapeutic opportunities in amyloidosis-related thoracic aortic diseases. Methods and Materials: The PRISMA Extension for Scoping Reviews (PRISMA-ScR) Guidelines were followed. An extensive search of the available literature in the English language, published between 1 January 2000, and 31 December 2025, in three large-scale scientific databases was undertaken by two independent reviewers. The terms "amyloidosis", "thoracic aorta", "thoracic aortic aneurysm", "aortic dissection", and "aortopathy" were used both as specific items, as well as in MeSH Terms. Studies reporting on the pathophysiology, diagnosis, clinical manifestations, treatment options and prognosis of amyloid deposition on the thoracic aorta were included in the review. Because of the nature of the existing literature, only a narrative review was possible. Results: Twenty-nine studies were included. Owing to the rarity of reporting, data was derived mainly from case reports and series, as well as autopsy studies. Amyloid infiltration of the aortic wall has been associated with medial architectural disruption, degeneration of elastic fibers, impairment of vasa vasorum perfusion, and arterial stiffness, all of which could contribute to aneurysmal dilation and aortic lesions. Amyloidosis management combines targeted anti-plasma cell therapy with supportive care. In AL amyloidosis, melphalan-dexamethasone (MDex) was historically standard, but regimens such as cyclophosphamide, bortezomib, and dexamethasone (CyBorD) and bortezomib, melphalan, and dexamethasone (BMDex) achieve higher complete response rates. Immunotherapy with Daratumumab has shown high overall and complete response rates. Fibril-directed approaches, including doxycycline and epigallocatechin gallate, and monoclonal antibodies, are under evaluation. Acute management of large-vessel manifestations follows conventional protocols, but prognosis is often dominated by underlying cardiac and systemic involvement. Conclusions: Management of thoracic aortic involvement follows standard imaging surveillance and surgical criteria, though operative risk is increased. Multidisciplinary care, early recognition, and individualized risk stratification are essential to improve outcomes, particularly given frequent cardiac involvement.
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2026-08-10 | IgM Pleural Amyloidosis Diagnosed by Pleural Effusion Cell Block: A Case Report.
Immunoglobulin light chain (AL) amyloidosis is characterized by the systemic deposition of light-chain-derived amyloids. Immunoglobulin M (IgM)-associated AL amyloidosis is extremely rare and clinically distinct. A 70-year-old man presented with dyspnea, bilateral pleural effusion, and mediastinal lymphadenopathy on computed tomography (CT). Amyloid was detected in a pleural effusion cell block and confirmed via an axillary lymph node biopsy. Serum studies and bone marrow findings supported IgM-λ AL amyloidosis associated with Waldenström macroglobulinemia/lymphoplasmacytic lymphoma. First-line therapy failed, but second-line therapy reduced pleural effusion, avoiding thoracentesis for one year. A pleural effusion cell block analysis enabled rapid diagnosis with minimally invasive exploration.
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2026-08-08 | Limited duration of dexamethasone in newly diagnosed AL amyloidosis: impact on response, toxicity, and survival.
Systemic light chain amyloidosis (AL) is a life-threatening disease in which dexamethasone (Dex) is a core therapy but is limited by cumulative toxicity. The optimal duration of Dex, particularly in the era of daratumumab (Dara)-based regimens, is uncertain. We retrospectively analyzed 216 newly diagnosed AL amyloidosis patients (2017-2023). Dex exposure was categorized as limited (≤3 months) or prolonged (>3 months) using restricted cubic spline-derived associations with hematologic response kinetics. Outcomes included hematologic and organ response, toxicity, and overall survival. Median Dex duration was 5.5 months and was similar by Dara use. Overall response rates exceeded 90% at 6 months in both Dex groups. Limited Dex was associated with higher rates of early deep hematologic response (VGPR or better 77.5 vs 56.5%, p = 0.001; CR 42.2 vs 19.4%, p < 0.001), reflecting faster response kinetics, while overall response rates were similar. Among patients receiving limited Dex, Dara was associated with higher 6-month CR rates (66.7 vs 30.4%, p < 0.001) and increased likelihood of achieving CR in multivariable analysis (HR 4.38, 95% CI 2.44-7.85; p < 0.001). Organ responses were similar between groups. Dex-related toxicities increased after 6 months, and hospitalization was associated with worse survival. Limiting Dex exposure was associated with preserved efficacy and reduced toxicity. Prolonged Dex was associated with increased toxicity without improving overall hematologic or organ outcomes, supporting limited Dex duration in frontline AL amyloidosis therapy.
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2026-08-08 | AL Cardiac Amyloidosis in Resource-Limited Settings: Diagnostic Challenges and Therapeutic Gaps.
Amyloid light chain (AL) cardiac amyloidosis remains a diagnostic challenge in resource-limited settings. We present 3 cases with varying heart failure presentations, all demonstrating concentric left ventricular hypertrophy with characteristic apical sparing strain pattern on echocardiography. Serum free light chains were consistently abnormal, though immunofixation was negative in 2 patients. Traditional screening biopsies were negative in 2 cases, requiring endomyocardial biopsy for diagnosis. Mass spectrometry for amyloid typing was unavailable; immunofluorescence performed in 1 patient confirmed lambda light chain deposition. All patients received CyBorD-based therapy; 1 patient additionally accessed daratumumab abroad and showed marked improvement, while another died within 6 months on CyBorD alone. These cases highlight the heterogeneous presentation of presumed AL amyloidosis and underscore how diagnostic barriers contribute to delayed diagnosis and more advanced disease at presentation, emphasizing the urgent need for improved diagnostic capabilities and therapeutic accessibility in resource-constrained settings.
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2026-08-03 | [Improvement of chylothorax after lymphangiography in AL amyloidosis].
A 74-year-old man presented with leg edema and was found to have nephrotic-range proteinuria. Renal biopsy revealed AL amyloidosis. Subsequent bone marrow examination demonstrated an increase in monoclonal plasma cells accounting for 13% of nucleated cells. Right-sided pleural effusion developed and was confirmed to be chylothorax. Although treatment with daratumumab, lenalidomide, and dexamethasone (DLd regimen) was initiated, the pleural effusion continued to increase. Lymphangiography using ethiodized oil identified a leakage point in the anterior mediastinum. A chest drain was inserted on the following day, and approximately 4 l of chylous fluid was drained. The tube was removed one week later, and pleural effusion did not recur thereafter. Although a hematologic response was achieved, the nephrotic syndrome persisted, and the patient ultimately died. Chylothorax associated with AL amyloidosis is rare and difficult to treat. This case suggests that lymphangiography using ethiodized oil may be both diagnostic and therapeutic.
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