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Diagnosis and treatment of multisystem amyloidosis associated with SGPL1 mutation: A case report and review of the literature.

RATIONALE: Amyloidosis is a rare, clinically heterogeneous disease, which makes its diagnosis difficult. The relationship between amyloidosis and gene mutations is insufficiently understood. We report a case of sphingosine-1-phosphate lyase 1 (SGPL1) mutation-related amyloidosis, and review the related literature. PATIENT CONCERNS: A 53-year-old man was admitted to our hospital with a 5-month history of renal dysfunction and abdominal distension, aggravated since 2 days. Five months ago, the patient was diagnosed with nephrotic syndrome, with liver dysfunction and abdominal distension. His sy

Diagnostic and therapeutic challenges in rapidly progressing cardiac amyloidosis: a literature review based on case report.

INTRODUCTION: Cardiac amyloidosis is a rarely reported and potentially fatal variant of the systemic disease. Its early diagnosis could potentially lead to significantly improved clinical outcomes. CASE PRESENTATION: A 56-year-old female presented with dyspnea and palpitations. Her physical exam and non-invasive evaluation with cardiac magnetic resonance imaging (CMRI) revealed restrictive cardiomyopathy, and the bone marrow biopsy results showed systemic amyloidosis. DISCUSSION: The diagnosis of cardiac amyloidosis is not always straightforward, and delay can cause the progression of the di

Extraskeletal multiple myeloma presenting with an atrial mass: a case report and a review of the literature.

INTRODUCTION: Extraskeletal presentation at diagnosis or during the course of multiple myeloma is a rare event. The prognosis is usually very poor. At the moment there is no agreed gold standard for the treatment of this presentation. CASE PRESENTATION: A 79-year-old Caucasian woman was treated at our hospital for right atrial myeloma localization. Our patient showed the following signs and symptoms of congestive heart failure: dyspnea, hypotension, cyanosis and facial edema. Surgery was not considered feasible due to the extent of the disease. Our patient underwent external-beam radiation th

Serious adverse events and coping strategies of CAR-T cells in the treatment of malignant tumors.

Chimeric antigen receptor T (CAR-T) cells technology has been successfully used in the treatment of B cell-derived hematological tumors and multiple myeloma. CAR-T cells are also being studied in a variety of solid tumors. Current clinical reports on CAR-T cells in the treatment of malignant tumors are abundant. The tumor-killing activity of CAR-T cells and the unique adverse effects of CAR-T cells have been confirmed by many studies. There is evidence that serious adverse events can be life-threatening. CAR-T cells therapy is increasingly used in clinical settings, so it is important to pay a

Analysis of factors influencing the increase of extracellular water ratio in tumor patients without edema signs.

ObjectiveThis study aims to examine the factors contributing to the increase in extracellular water to total body water ratio (ECW/TBW) among tumor patients without visible edema, and to offer insights into the diagnosis and prompt management of early water retention in such patients.MethodsA cohort of 274 tumor patients admitted to Anqing First People's Hospital, affiliated with Anhui Medical University between December 2020 and December 2022, were selected, excluding those with clinically observable edema. General clinical data, main laboratory test outcomes, and body composition indices wer

Post-transplant cyclophosphamide-induced cardiotoxicity: A comprehensive review.

Cyclophosphamide-induced cardiotoxicity, associated with its toxic metabolite acrolein, is a significant concern and unresolved issue, especially when cyclophosphamide is administrated in high doses. However, cardiotoxicity following low-dose cyclophosphamide has been also documented, especially in post-hematopoietic stem cell transplantation (post-HSCT) settings. Despite the involvement of multiple signaling pathways in cyclophosphamide-induced cardiomyopathy, the exact underlying mechanisms remain to be fully elucidated. This review outlines the current challenges of cyclophosphamide therapy

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… The most observed complications observed among cyclophosphamide recipients were acute pulmonary edema, cardiac arrest, and cardiac arrhythmia, respectively. 42 The most common echocardiography findings were also related to systolic dysfunction and pericardial effusion in this subset of patients. 42 The …

Toxicity of CAR T-Cell Therapy for Multiple Myeloma.

BackgroundIdecabtagene vicleucel (ide-cel) and ciltacabtagene autoleucel (cilta-cel) are novel chimeric antigen receptor (CAR)-T cell therapies targeting B-cell maturation antigen (BCMA), and both have recently gained approval by the US Food Drug Administration (FDA) for the treatment of relapsed and refractory multiple myeloma (RRMM).SummaryThese therapies offer unprecedented responses in RRMM but present new challenges including cytokine release syndrome (CRS), immune effector cell-associated neurotoxicity syndrome (ICANS), non-ICANS neurotoxicity, cytopenias, infections, and hypogammaglobul

Cardiovascular adverse events associated with targeted therapies for multiple myeloma: a pharmacovigilance study.

IntroductionMultiple myeloma (MM) is a leading cause of hematopoietic cancer-related mortality, accounting for 20% of deaths. MM-targeted therapies have demonstrated efficacy, and since 2015, the United States Food and Drug Administration (FDA) has approved five targeted drugs. However, their cardiovascular safety has not been comprehensively evaluated.ObjectiveThis study aimed to investigate the association between MM-targeted therapy and cardiovascular adverse events (AEs).MethodsDisproportionality analysis was conducted on reports from the FDA AE Reporting System database from 2014 to the s

Amyloidosis with Cardiac Involvement: Identification, Characterization, and Management.

Purpose of reviewAmyloidosis is a protein deposition disease whereby a variety of precursor proteins form insoluble fibrils that deposit in tissues, causing organ dysfunction and, many times, death. Accurate characterization of the disease based on the nature of the precursor protein, organ involvement, and extent of disease is paramount to guide management. Cardiac amyloidosis is critical to understand because of its impact on prognosis and new treatment options available.Recent findingsNew imaging methods have proven to be considerably valuable in the identification of cardiac amyloid infilt

Navigating the Diagnostic and Management Challenges of Suspected Cardiac Amyloidosis in Resource-Limited Settings: Ethiopia Experience.

This report discusses the diagnostic and therapeutic complexities encountered in resource-limited environments when assessing patients suspected of cardiac amyloidosis (CA). The study describes a case involving a patient who initially presented with heart failure symptoms and eventually received a CA diagnosis after 13 months and four primary physician visits, primarily based on discrepancies observed between electrocardiographic and echocardiographic findings, alongside elevated cardiac troponin levels. The case underscores the limited awareness of CA among primary healthcare providers in Eth

Uncovering the knowledge about systemic amyloidosis relevant to the rheumatologists.

Amyloidosis is a localized or systemic disease caused by deposition of proteins in the extracellular space of various organs and tissues. As part of the disease, proteins that were originally soluble misfold and acquire a fibrillar conformation that renders them insoluble and resistant to proteolysis. Systemic amyloidosis is a rare, often underdiagnosed condition. In recent years, the incidence of newly diagnosed cases of amyloidosis has been increasing in association with the aging of the population and greater access to diagnostic tests. From a clinical perspective, systemic amyloidosis is f

Dermatological Manifestations of Amyloid Light-Chain (AL) Amyloidosis: A Case Report Highlighting Early Diagnosis and Treatment.

This case report describes a 52-year-old man who presented with gradually progressive dark cutaneous discoloration over the upper trunk and periorbital region, accompanied by painless enlargement of the tongue, which initially interfered with speech and eating. Over the subsequent months, he experienced marked unintentional weight loss, persistent fatigue, and symptoms consistent with autonomic involvement, including postural dizziness and altered bowel habits. Clinical examination revealed waxy skin changes and macroglossia. Laboratory evaluation demonstrated a monoclonal light-chain abnormal

Supportive Care and Symptom Management for Patients With Immunoglobulin Light Chain (AL) Amyloidosis.

Immunoglobulin light chain (AL) amyloidosis is a disorder of clonal plasma cells characterized by deposition of amyloid fibrils in a variety of tissues, leading to end-organ injury. Renal or cardiac involvement is most common, though any organ outside the central nervous system can develop amyloid deposition, and symptomatic presentations may consequently vary. The variability and subtlety of initial clinical presentations may contribute to delayed diagnoses, and organ involvement is often quite advanced and symptomatic by the time a diagnosis is established. Additionally, while organ function

Cardiac Amyloidosis Treatment.

Cardiac amyloidosis (CA) is a restrictive cardiomyopathy with a traditionally poor prognosis. Until recently, CA treatment options were limited and consisted predominantly of managing symptoms and disease-related complications. However, the last decade has seen significant advances in disease-modifying therapies, increased awareness of CA, and improved diagnostic methods resulting in earlier diagnoses. In this review, we provide an overview of current and experimental treatments for the predominant types of CA: transthyretin cardiac amyloidosis (ATTR-CA) and immunoglobulin light chain (AL)-med

Novel Therapeutics for Anthracycline Induced Cardiotoxicity.

Anthracyclines remain an essential component of the treatment of many hematologic and solid organ malignancies, but has important implications on cardiovascular disease. Anthracycline induced cardiotoxicity (AIC) ranges from asymptomatic LV dysfunction to highly morbid end- stage heart failure. As cancer survivorship improves, the detection and treatment of AIC becomes more crucial to improve patient outcomes. Current treatment modalities for AIC have been largely extrapolated from treatment of conventional heart failure, but developing effective therapies specific to AIC is an area of growing

Untangling the clinical and economic burden of hospitalization for cardiac amyloidosis in the United States.

PurposeCardiac dysfunction is common in amyloid light-chain (AL) amyloidosis, a rare disease caused by extracellular deposition of misfolded immunoglobulin light chains. This study aimed to examine economic/clinical disease burden in hospitalized cardiac amyloidosis patients.Patients and methodsCardiac amyloidosis patients ≥18 years old hospitalized between 2014 and 2016 were identified in claims if they had ≥1 inpatient claim consistent with amyloidosis and evidence of cardiac dysfunction. Descriptive statistics were reported.Results3239 cardiac amyloidosis patients [1795 (55.4%) with concurr

Amyloidosis: A Rare Cause of Severe Cholestasis and Acute Liver Failure.

Although hepatic involvement in light chain-associated amyloidosis is common, clinical manifestations of hepatic amyloidosis are rare. In most cases, hepatomegaly serves as a clue to diagnosis. We report a unique case of a 48-year-old man from China with jaundice and noncirrhotic portal hypertension, with rapidly progressive liver failure, in the absence of hepatomegaly, secondary to systemic light chain-associated amyloidosis associated with multiple myeloma.

Cardiac Monitoring for Patients Undergoing Treatment With MEK Inhibitor Monotherapy.

PURPOSE OF REVIEW: Aberrant activation of the RAS-RAF-MEK-ERK signaling pathway contributes to numerous malignancies, congenital syndromes, and cardiovascular disorders, establishing MEK inhibitors (MEKi) as crucial therapeutic agents. MEKi demonstrate substantial clinical benefit as monotherapy (e.g., NF1-associated tumors) or combination therapy (e.g., melanoma, glioma, congenital RASopathies). Cardiovascular adverse events, primarily asymptomatic reductions in left ventricular ejection fraction (LVEF), have prompted intensive echocardiographic monitoring. RECENT FINDINGS: Emerging evidence

The pathogenesis, diagnosis, prevention, and treatment of CAR-T cell therapy-related adverse reactions.

Chimeric antigen receptor (CAR)-T cell therapy is effective in the treatment of refractory/relapsed (r/r) hematological malignancies (r/r B-cell lymphoblastic leukemia, B-cell lymphoma, and multiple myeloma). In addition, it is being explored as a treatment option for solid tumors. As of 31 March 2022, seven CAR-T therapies for hematological malignancies have been approved worldwide. Although CAR-T therapy is an effective treatment for many malignancies, it also causes adverse effects. The incidence of cytokine release syndrome (CRS), the most common adverse reaction after infusion of CAR-T ce

Beyond fluid overload: uncovering the hidden causes of dyspnea in hemodialysis patients.

BACKGROUND: Shortness of breath is a common and complex symptom in patients with advanced kidney failure treated with hemodialysis. It may result from excess fluid accumulation, cardiac dysfunction, or impaired lung performance. We studied the interactions between hydration status, pulmonary function, and muscle strength. METHODS: This cohort study included 29 patients on maintenance hemodialysis. Each participant underwent lung function testing, diffusing capacity for carbon monoxide measurement, handgrip strength assessment, body composition analysis by bioimpedance, echocardiography, and l