Primary (AL) amyloidosis in plasma cell disorders.
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.
Müller
Antonia M S
AM
Department of Hematology/Oncology, University Medical Center Freiburg, Hugstetterstr. 55, D-79106 Freiburg, Germany.
Geibel
Annette
A
Neumann
Hartmut P H
HP
Kühnemund
Alexander
A
Schmitt-Gräff
Annette
A
Böhm
Joachim
J
Engelhardt
Monika
M
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
D007147:Immunoglobulin Light Chains