Amyloid and Related Disorders

Maria M. Picken, MD, PhDProfessor of PathologyDirector of Surgical PathologyPast President Renal Pathology SocietyLoyola University Medical CenterDepartment of Pathology2160 South First Avenue, Building 110, Room 2242Maywood, ILUSAGuillermo A. Herrera, M.D.Albert G. And Harriet G. Smith Professor and ChairLSU Health ShreveportDepartment of Pathology1501 Kings HighwayShreveport, LA  USAAhmet Dogan, MD, PhDChief, Hematopathology ServiceMemorial Sloan Kettering Cancer CenterDepartment of Pathology and Laboratory Medicine1275 York AvenueNew York, NY  USA

Introduction/general.- Aspects of the history and nomenclature of amyloid and amyloidosis.- Amyloid diseases at the molecular level: general overview and focus on AL amyloidosis.- AA amyloidosis.- Non-Immunoglobulin and non-AA amyloidoses.- The hereditary amyloidoses.- Dialysis-associated amyloidosis.- Localized amyloidoses and amyloidoses associated with aging outside the central nervous system.- Central nervous system amyloid.- Non-amyloid protein deposits.- Differential diagnosis of amyloid in surgical pathology: organized deposits and other material in the differential diagnosis of amyloidosis.- Light /heavy chain deposition disease as a systemic disease.- Glomerulonephritis with non-organized monoclonal immunoglobulin deposits.- Pathologies of renal and systemic intracellular paraprotein storage: crystalopathies and beyond.- Diagnosis.- Diagnosis of amyloid using Congo red - polarized and fluorescence.- Thioflavin T stain for amyloid detection.- Fat tissue analysis in the management of patients with systemic amyloidosis.- Generic diagnosis of amyloid - recent developments and summary of current recommendations.- Amyloid typing on formalin-fixed paraffin sections by immunohistochemistry - experience from two large referral centers.- Options for amyloid typing in renal pathology - the advantages of frozen section immunofluorescence.- Amyloid typing by immuno-electron microscopy.- Classification of amyloidosis by mass spectrometry-based proteomics.- Summary and practical recommendation regarding amyloid typing using antibody-based and proteomic methods.- Ancillary studies of amyloidosis.- Laboratory support for diagnosis of amyloidosis.- Bone marrow biopsy and its utility in the diagnosis of AL amyloidosis and other plasma cell dyscrasias.- Molecular methods for the diagnosis of hereditary amyloidosis.- Radiologic support for diagnosis and monitoring of amyloidosis - established techniques and recent developments.- Organ involvement inamyloidoses.- Amyloidoses of the Kidney and Genitourinary tract.- Cardiac amyloidosis.- Amyloidosis of the Gastrointestinal tract and Liver.- Peripheral nerve amyloidosis.- Amyloidoses of the Pulmonary and upper respiratory tract.- Amyloidoses involving the skin.- Clinical issues and therapy.- Clinico-pathologic issues in patients with amyloidosis.- Solid organ transplantation in amyloidosis and issues pertaining to domino liver transplantation.- Emerging therapies for amyloidosis.- Clinical trials.- Modern therapies in AA amyloidosis.- Medico legal issues of amyloidosis.- Amyloidosis from the patient's perspective.