Non-light-chain immunoglobulin amyloidosis: time to expand or refine the spectrum to include light+heavy chain amyloidosis?
نویسندگان
چکیده
منابع مشابه
Atypical immunoglobulin light chain amyloidosis
BACKGROUND Primary immunoglobulin light chain amyloidosis (AL amyloidosis) is a plasma cell disorder which mainly affects heart, kidneys, liver, and peripheral nervous system. Cases of atypical AL amyloidosis presented as spontaneous vertebral compression fractures have been rarely reported, and data about the management and clinical outcomes of the patients are scarce. METHODS Herein, we pre...
متن کاملHereditary systemic immunoglobulin light-chain amyloidosis.
Several members of a family died from renal failure as a result of systemic amyloidosis. Extensive studies to detect previously documented gene mutations associated with amyloidosis failed to identify a causative factor. In search of the genetic basis for this syndrome, amyloid fibrils were isolated from renal tissue of a member of the kin who died while on renal dialysis. Amino acid sequencing...
متن کاملTreatment of immunoglobulin light chain (primary or AL) amyloidosis.
Not all forms of amyloidosis are systemic. Some patients may present with a localized form and should not be treated with chemotherapy. Some patients with systemic amyloidosis may have secondary, familial, or dialysis-related types.These types are not responsive to chemotherapy. Immunoglobulin light chain (primary or AL) amyloidosis is a plasma cell dyscrasia. Suppression of light chain product...
متن کاملImmunoglobulin heavy-chain-associated amyloidosis.
Immunoglobulin- or multiple myeloma-associated amyloidosis has been distinguished by the tissue deposition of Congophilic, fibrillar protein consisting of light chains or light-chain fragments (AL amyloidosis). We now report the isolation and characterization of another form of immunoglobulin-associated amyloid obtained from a patient who had extensive systemic amyloidosis and in whom the amylo...
متن کاملLight Chain Amyloidosis
Light chain (AL) amyloidosis is caused by a usually small plasma-cell clone that is able to produce the amyloidogenic light chains. They are able to misfold and aggregate, deposit in tissues in the form of amyloid fibrils and lead to irreversible organ dysfunction and eventually death if treatment is late or ineffective. Cardiac damage is the most important prognostic determinant. The risk of d...
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ژورنال
عنوان ژورنال: Kidney International
سال: 2013
ISSN: 0085-2538
DOI: 10.1038/ki.2012.433