Introduction Hodgkin's disease may be associated with a variety of pathological lesions in the liver other than lymphomatous infiltration; lesions include granulomas, cholestasis which may rarely progress to cirrhosis, amyloidosis, and portal tract accumulations of histiocytes, eosinophils, lymphocytes and granulocytes. The patient to be reported, and several previous reports, suggest that sinu...

Background Transthyretin related familial amyloidotic polyneuropathy (TTR-FAP) is an inherited form of amyloidosis, leading to death in about 10 years in most cases for cardiac failure or wasting syndrome. Previous study showed that modified body mass index (mBMI) was related to time before death, duration of gastrointestinal disturbances, malabsorption and functional capacity. Futhermore, outc...

A 49 years old lady presented with low-grade fever (99-100°F) for 2 years. During this time she was extensively worked-up for pyrexia of unknown origin but no diagnosis could be established. Her Initial blood work-up was all negative except high alkaline phosphatase and gamma GT (374 IU and 195 IU respectively). She later presented to our tertiary care centre with facial swelling, flushing and ...

Spanish Ministry of Economy and Competitiveness (grant N BFU 2012-36241) and Programa INNPACTO (grant N IPT-2011-0817-010000) (to Dr. Martin), and from the Agence Nationale de la Recherche (Programme Blanc BCNCT) (to Dr. Lambert). Dr. Alves was a recipient of a PhD grant SFRH/BD/27990/2006 and research grant PTDC/SAU-GMG/101874/2008 from FCT. Mrs. Medeiros was supported by a research grant from...

INTRODUCTION Leukocyte cell-derived chemotaxin-2 (LECT2) is a highly conserved protein with roles in cell development, sepsis, liver disease, metabolic syndrome, arthritis, cancer, and amyloidosis, among others. We will review its genetics, structure, role in physiology, and its future as a biomarker and therapeutic target across many diseases. Studies involving LECT2 were identified using PubM...

a Wolfson Drug Discovery Unit, Centre for Amyloidosis and Acute Phase Proteins, Division of Medicine, University College London, London NW3 2PF, UK; b Centre for Biomedical Science, Division of Medicine, University College London, London NW3 2PF, UK; Department of Molecular Medicine, University of Pavia, 27100 Pavia, Italy; d Centre for Neuroendocrinology, Division of Medicine, University Colle...

Sections of liver and spleen from six cases of secondary amyloidosis have been examined, by various improved histochemical methods, for acidic mucosubstances. The results indicate a complex in amyloid deposits of protein and sulphated mucopolysaccharides in which the principal substance is heparan sulphate with traces of chondroitin sulphates. Deposits also contain sialic acid. The results are ...

Familial amyloid polyneuropathy type I (FAP type I) is a rare hereditary systemic amyloidosis caused by the Val30Met mutation in the transthyretin (TTR) gene. The clinical onset and spectrum are variable and depend on phenotypic heterogeneity. Cardiac complications (dysrhythmias and conduction disturbances, cardiomyopathy and dysautonomia) indicate a poor prognosis, even after liver transplanta...

AIMS Familial amyloid polyneuropathy (FAP) is a dominantly inherited multi-system disease associated with transthyretin (TTR) mutations. Previous series have predominantly described patients with the TTR variant Val30Met (V30M), which is the most prevalent cause of FAP worldwide. Here, we report the dominant cardiac phenotype and outcome of FAP associated with TTR Thr60Ala (T60A), the most comm...

Amyloidosis is a rare disorder characterised by the deposition of abnormal protein fibrills in various tissues and organs.In context multiple myeloma,a plasma cell dyscrasia amyloidosis can occur as distinct complication, referred to myeloma associated amyloidosis.