title: Primary (AL) amyloidosis in plasma cell disorders.
abstract: Primary (AL) amyloidosis is the most common form of systemic amyloidosis. The morbidity arises from extracellular deposition of immunoglobulin light chain (LC) fibrils in major organs, such as the kidneys, heart, and bowel. Organ dysfunction contributes to a high mortality and poor prognosis, with a median survival time of 1-2 years from diagnosis. Here, we present a 46-year-old man with an exceptional clinical course of an LC multiple myeloma with generalized amyloidosis, causing renal insufficiency, congestive heart failure, and complete intestinal necrosis. We have summarized recent knowledge on AL amyloidosis, its association with monoclonal gammopathies, clinical presentations, diagnostic tools, and treatment strategies. Our comprehensive overview of this rare and often fatal disease aims to increase the awareness of AL amyloidosis. This may facilitate earlier diagnosis, and thus allow initiation of prompt and specific therapies, which are indispensable in order to improve disease prognosis.
authors.affiliation: Department of Hematology/Oncology, University Medical Center Freiburg, Hugstetterstr. 55, D-79106 Freiburg, Germany.
authors.forename: Antonia M S
authors.initials: AM
authors.lastname: Müller
authors.forename: Annette
authors.initials: A
authors.lastname: Geibel
authors.forename: Hartmut P H
authors.initials: HP
authors.lastname: Neumann
authors.forename: Alexander
authors.initials: A
authors.lastname: Kühnemund
authors.forename: Annette
authors.initials: A
authors.lastname: Schmitt-Gräff
authors.forename: Joachim
authors.initials: J
authors.lastname: Böhm
authors.forename: Monika
authors.initials: M
authors.lastname: Engelhardt
mesh_terms: D000686:Amyloidosis / Q000150:complications* / Q000276:immunology / Q000628:therapy; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications* / Q000276:immunology / Q000628:therapy; D009336:Necrosis; D010950:Plasma Cells / Q000473:pathology*; D010956:Plasmapheresis; D011379:Prognosis; D006435:Renal Dialysis; D051437:Renal Insufficiency / Q000209:etiology
chemical_list: D007147:Immunoglobulin Light Chains