Light-Chain (AL) Amyloidosis

Abstract

Light-chain (AL) amyloidosis is the most common form of systemic amyloidosis and is associated with an underlying plasma cell dyscrasia. The disease often is difficult to recognize because of its broad range of manifestations and what often are vague symptoms. The clinical syndromes at presentation include nephrotic-range proteinuria with or without renal dysfunction, hepatomegaly, congestive heart failure, and autonomic or sensory neuropathy. Recent diagnostic and prognostic advances include the serum free light-chain assay, cardiac magnetic resonance imaging, and serologic cardiac biomarkers. Treatment strategies that have evolved during the past decade are prolonging survival and preserving organ function in patients with this disease. This review outlines approaches to diagnosis, assessment of disease severity, and treatment of AL amyloidosis.

Epidemiology of AL amyloidosis: a real-world study using US claims data

“…Cardiac dysfunction commonly manifests as heart failure. 3 Renal involvement usually presents as nephrotic syndrome with progressive worsening of renal functions.…”

The importance of macroglossia and the enlargement of the submandibular salivary glands for the diagnostics of AL-amyloidosis in the practical work of a dental practitioner. A case report and review of the literature

“…Постановка диагноза AL-амилоидоза вызывает затруднения у врачей во всем мире в связи с разно-образием клинических проявлений и неопределен-ными симптомами [9,[11][12][13]. Значительное увели-чение ПЧСЖ встречается при аутоиммунных, гра-нулематозных, лимфопролиферативных и IgG4-связанных заболеваниях (см.…”

“…Наличие не-большого уровня моноклональной секреции или легких цепей моноклональных Ig в сыворотке или моче является обязательным для AL-амилоидоза [9][10][11][12][13][14], тогда как отсутствие этих признаков позво-ляет предположить проявления вторичного AA-амилоидоза [5]. Учитывая многообразие диагнозов, которые были поставлены пациентке в течение го-да, следует остановиться на алгоритме обследования пациента, обратившегося в стоматологическую кли-нику с увеличением слюнных желез (см.…”

D-Dimer: A Novel Predictor of Survival in Patients with Cardiac Light- Chain Amyloidosis

“…AL amyloidosis is usually associated with plasma cell dyscrasia [2], in which a malignant proliferation of plasma cells secretes an unstable light chain that is prone to misfolding. AL amyloidosis is a relatively rare disease, occurring with an incidence of approximately 10 patients per million per year; however, cardiac involvement has been reported in approximately 70% [3] of cases [4], resulting in rapidly progressive heart failure (HF) and a very poor prognosis, especially in untreated patients.…”