Solitary pleural amyloid nodules occurring as coin lesions diagnosed by fine-needle aspiration biopsy

Solitary pleural amyloid nodule is rare and radiologically can mimic neoplastic lesions. This report describes two cases of this entity diagnosed by fine-needle aspiration biopsy in patients without systemic amyloid deposits. The histologic, ultrastructural, and cytologic features are described.     

Cardiac arrest in a patient with a mobile right atrial thrombus in transit and amyloidosis

Primary amyloidosis is a rare disorder in which insoluble fibersare deposited in tissue and organs, impairing their function.Cardiac involvement occurs in up to 50% of patients with primaryamyloidosis. We describe a case of a 75-year-old admitted to our departmentafter he had a sudden cardiac arrest due to massive bilateralthrombotic occlusion of the pulmonary arteries. The echocardiogramrevealed many atrial thrombi swirling inside the right atriumand protruding into the tricuspid valve partly occluding it.Severe concentric hypertrophy of the left ventricle was alsopresent with a preserved ejection fraction. The right ventriclewas dilated, hypertrophic and ipokinetic with a severe tricuspidalinsufficiency that permitted estimation of a severe pulmonaryhypertension. All these characteristics were highly suggestivefor an infiltrative form of hypertrophic cardiomyopathy. Thefinal diagnosis was amyloidosis.     

Castleman disease with renal amyloidosis: imaging findings and clinical significance

We report two cases of renal amyloidosis associated with mesenteric and mediastinal Castleman disease of plasma cell type. Computed tomographic (CT) scan revealed a mesenteric mass with multiple nodal enlargement in one patient and a right paratracheal mass with irregular central calcifications in the second patient. Renal ultrasonography (US) showed increased echogenicity of the renal parenchyma with prominent medullas. Doppler waveform obtained in the kidney associated with mesenteric disease showed complete absence of diastolic flow signal, although it was normal in the patient with mediastinal disease. Recognition of Castleman disease as the cause of renal amyloidosis and the understanding of CT and US findings of this entity are important for the management, as well as its diagnosis.     

Adverse Prognostic Factors for Morbidity and Mortality During Peripheral Blood Stem Cell Mobilization in Patients with Light Chain Amyloidosis

Patients with immunoglobulin light chain (AL) amyloidosis undergoing peripheral blood hematopoietic stem cell (PBSC) mobilization for autologous hematopoietic stem cell transplantation (auto-HCT) can experience significant morbidity and mortality. The purpose of this study was to characterize the adverse events and identify prognostic factors associated with the development of morbidity and mortality in patients with AL amyloidosis who had begun PBSC mobilization for auto-HCT. A retrospective study was performed in 101 consecutive patients with AL amyloidosis who underwent PBSC mobilization for auto-HCT between January 2006 and December 2013. A composite primary endpoint of morbidity and mortality during PBSC mobilization was used. Forty-one patients (41%) experienced at least 1 adverse event, including 4 deaths during PBSC mobilization. Adverse events included in this composite endpoint were cardiac events, thromboembolic events, bleeding events, unplanned hospitalization, weight gain?>2% necessitating diuretic intervention, and death. Low serum albumin levels, elevated N-terminal pro-brain natriuretic peptide, and increased interventricular septal thickness were significantly associated with the composite primary endpoint (P?=?.024, .001, and .006, respectively). The median progression-free survival from the start of PBSC mobilization was 4.7 years, and the median overall survival was 6.5 years. In general, PBSC mobilization is associated with minimal complications, but patients with AL amyloidosis can experience more frequent and severe complications, such as volume overload and weight gain. Careful patient selection is warranted in patients with AL amyloidosis before proceeding to PBSC mobilization and auto-HCT.     

Localized amyloidosis of the stomach： A case report

We report an unusual case of primary amyloidosis of the stomach in a patient complaining of dyspeptic symptoms. The diagnosis was confirmed histologically and other gastrointestinal site or systemic involvement was ruled out, Uncharacteristic dyspeptic symptoms may hide this rare metabolic disease.     

A hidradenitis suppurativa related SAPHO case associated with features resembling spondylarthropathy and proteinuria

We present a 53-year-old man with synovitis–acne–palmoplantar pustulosis–hyperosteosis–osteitis (SAPHO) syndrome who is HLA-B27 positive with a history of uveitis and complicated by proteinuria and osteoporosis. Interesting, yet unreported features of SAPHO syndrome and the etiology of proteinuria are further discussed.     

Human apolipoprotein A-I Gly26Arg stimulation of inflammatory responses via NF-kB activation: Potential roles in amyloidosis?

The cascade of molecular events leading to Human apolipoprotein A–I (apoA–I) amyloidosis is not completely understood, not even the pathways that determine clinical manifestations associated to systemic protein deposition in organs such as liver, kidney and heart. About twenty natural variants of apoA–I were described as inducing amyloidosis, but the mechanisms driving their aggregation and deposition are still unclear. We previously identified that the mutant Gly26Arg but not Lys107-0 induced the release of cytokines and reactive oxygen species from cultured RAW 264.7 murine macrophages, suggesting that part of the pathogenic pathway could elicit of an inflammatory signal. In this work we gained deep insight into this mechanism and determined that Gly26Arg induced a specific pro-inflammatory cascade involving activation of NF-κB and its translocation into the nucleus. These findings suggest that some but not all apoA–I natural variants might promote a pro-oxidant microenvironment which could in turn result in oxidative processing of the variants into a misfolded conformation.