Neoplastic Process
primary amyloidosis
pri·ma·ry am·y·loi·do·sis
Subclass of:
Lymphoproliferative Disorders;
Plasma Cell Neoplasm;
Paraproteinemias;
Amyloidosis
Definitions related to primary amyloidosis:
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(al amyloidosis) A plasma cell disorder characterized by the aggregation and deposition of insoluble amyloid fibrils derived from misfolding of monoclonal immunoglobulin light chains usually produced by a plasma cell tumor. It usually presents as primary systemic amyloidosis (PSA) with multiple organ involvement and less frequently as primary localized amyloidosis (PLA) restricted to a single organ.OrphaNetINSERM, 2021
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(al amyloidosis) The most common type of amyloidosis. It is characterized by the monoclonal deposition of immunoglobulin light chain fragments in organs and tissues. It is associated with plasma cell or B-cell lymphoproliferative disorders.NCI ThesaurusU.S. National Cancer Institute, 2021
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A plasma cell neoplasm that secretes an abnormal immunoglobulin, which deposits in tissues and forms a beta-pleated sheet structure that binds Congo red dye with characteristic birefringence. (WHO, 2001)NCI ThesaurusU.S. National Cancer Institute, 2021
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(immunoglobulin light-chain amyloidosis) A nonproliferative disorder of the PLASMA CELL characterized by excessive production and misfolding of IMMUNOGLOBULIN LIGHT CHAINS that form insoluble amyloid fibrils in various tissues. Clinical features include LIVER FAILURE; MULTIPLE MYELOMA; NEPHROTIC SYNDROME; RESTRICTIVE CARDIOMYOPATHY, and neuropathies.NLM Medical Subject HeadingsU.S. National Library of Medicine, 2021
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AL amyloidosisis the most common form of amyloidosis, a group of disorders in which an abnormal protein called amyloid builds up in tissues and organs. The signs and symptoms of AL amyloidosis vary among patients because the build up may occur in the tongue, intestines, muscles, joints, nerves, skin, ligaments, heart, liver, spleen, or...National Center for Advancing Translational Sciences
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