Cardiac Amyloid Detection by PET/CT Imaging of Iodine (I) Evuzamitide (I-p5+14): A Phase 1/2 Study.

Background: The noninvasive detection of cardiac amyloid, as well as deposits in other vital organs, is critical for early diagnosis and quantitative disease monitoring. Positron emission tomography is an intrinsically quantitative imaging modality suitable for high-resolution amyloid detection.

Objectives: This study sought to evaluate the safety and efficacy of a novel amyloid-reactive peptide, designated p5+14, labeled with iodine-124 (I), in patients with diverse types of systemic amyloidosis.

Thromboembolic Rates Are Similar Between Intensive Care Unit and Nonintensive Care Unit Hospitalized Patients With Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2): A Retrospective Cohort Study.

High rates of thromboembolic events have been described in intensive care unit (ICU) patients. Data regarding thromboembolic events in all hospitalized patients has been less frequently reported, raising concerns that thromboembolic events in non-ICU may be underrecognized. In addition, optimal anticoagulation type and dose is still unsettled at this time.

First in Human Evaluation and Dosimetry Calculations for Peptide I-p5+14-a Novel Radiotracer for the Detection of Systemic Amyloidosis Using PET/CT Imaging.

Purpose: Accurate diagnosis of amyloidosis remains a significant clinical challenge and unmet need for patients. The amyloid-reactive peptide p5+14 radiolabeled with iodine-124 has been developed for the detection of amyloid by PET/CT imaging. In a first-in-human evaluation, the dosimetry and tissue distribution of I-p5+14 peptide in patients with systemic amyloidosis.

Hereditary Hyperferritinemia-Cataract Syndrome in 3 Generations of a Family in East Tennessee.

Hereditary hyperferritinemia and cataracts syndrome (HHCS) without iron overload is a syndrome first identified less than 3 decades ago. While investigators have dissected the gene where several responsible mutations reside, it remains a relatively unknown genetic disorder to clinicians. The result is often an expensive, invasive evaluation for iron overload, followed by a well-intended prescription for a series of phlebotomies that delivers morbidity instead of benefit.

Bifunctional amyloid-reactive peptide promotes binding of antibody 11-1F4 to diverse amyloid types and enhances therapeutic efficacy.

Amyloidosis is a malignant pathology associated with the formation of proteinaceous amyloid fibrils that deposit in organs and tissues, leading to dysfunction and severe morbidity. More than 25 proteins have been identified as components of amyloid, but the most common form of systemic amyloidosis is associated with the deposition of amyloid composed of Ig light chains (AL). Clinical management of amyloidosis focuses on reducing synthesis of the amyloid precursor protein.

A functional assay to identify amyloidogenic light chains.

Introduction: Multiple myeloma (MM) and light chain monoclonal gammopathy of undetermined significance (LCMGUS) are plasma cell disorders associated with the secretion of monoclonal free light-chain (LC) proteins. Due to the high concentrations of LC in circulation, both of these populations are at risk for developing LC-associated amyloidosis (AL) - a protein misfolding disease characterized by the deposition of LC protein fibrils in organs and tissues, leading to dysfunction and significant morbidity. At present, accurate identification of subjects at risk for developing amyloidosis is not possible, but with the advent of novel, amyloid-targeted therapies, identification of pre-symptomatic individuals is of clinical import.

Differential recruitment efficacy of patient-derived amyloidogenic and myeloma light chain proteins by synthetic fibrils-A metric for predicting amyloid propensity.

Background: Monoclonal free light chain (LC) proteins are present in the circulation of patients with immunoproliferative disorders such as light chain (AL) amyloidosis and multiple myeloma (MM). Light chain-associated amyloid is a complex pathology composed of proteinaceous fibrils and extracellular matrix proteins found in all patients with AL and in ~10-30% of patients who presented with MM. Amyloid deposits systemically in multiple organs and tissues leading to dysfunction and ultimately death.

Secondary Hemochromatosis due to Chronic Oral Iron Supplementation.

Iron may accumulate in excess due to a mutation in the HFE gene that upregulates absorption or when it is ingested or infused at levels that exceed the body's ability to clear it. Excess iron deposition in parenchymal tissue causes injury and ultimately organ dysfunction. Diabetes mellitus and hepatic cirrhosis due to pancreas and liver damage are just two examples of diseases that result from iron overload.

Partial Splenic Embolization as a Bridge to Total Knee Replacement for a Patient with Severe Thrombocytopenia due to Cirrhosis and Splenic Sequestration.

Medical splenectomy by embolization was originally used to attenuate bleeding from varices in a man with cirrhosis and portal hypertension. Despite the procedure being described over 40 years ago with remarkable improvement in its safety profile and clinical outcomes since, it is still used with variable frequency because of concerns that the risk is high and the results are transient. We present the case of an elderly woman with cirrhosis, portal hypertension, and splenic sequestration who completed partial splenic embolization (PSE) with a durable hematologic response that served as a bridge which allowed her to have orthopedic surgery.

Renal cell carcinoma and autoimmune hemolytic anemia.

A previously healthy man who became bedridden because of malaise, fatigue, and weakness was found to have an autoimmune hemolytic anemia (AIHA). In the course of his evaluation for the AIHA, he was found, coincidentally, to have a renal cell carcinoma. The AIHA was marginally responsive to therapy with corticosteroids, but it resolved promptly after excision of the cancer.

Non-Hodgkin's lymphoma originating in the spermatic cord.

An otherwise healthy 57-year-old man was found to have an early stage, high-grade, non-Hodgkin's lymphoma (NHL) of the spermatic cord. A plan of treatment involving surgery, radiation therapy, combination chemotherapy, and central nervous system prophylaxis was recommended. He did not complete the recommended treatment plan, and subsequently returned with recurrent tumor in his brain.