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Related terms:
amyloidosis
cryoglobulinemia
mononucleosis
hairy cell leukemia
lymphocytic leukemia
lymphangiomyoma
lymphoma
lymphoproliferative disorder
Marek's disease
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:
  • (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.
    OrphaNet
    INSERM, 2021
  • (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
    U.S. National Cancer Institute, 2021
  • Amyloidosis caused by monoclonal deposition of immunoglobulin light chain and, rarely, heavy chain fragments in organs and tissues. It is associated with plasma cell or B-cell lymphoproliferative disorders.
    NCI
    U.S. National Cancer Institute, 2021
  • (immunoglobulin light-chain amyloidosis) A nonproliferative disorder of PLASMA CELLS characterized by excessive production and misfolding of IMMUNOGLOBULIN LIGHT CHAINS that form insoluble amyloid fibrils (see AMYLOID DEPOSITS) in various tissues. Clinical features include LIVER FAILURE; MULTIPLE MYELOMA; NEPHROTIC SYNDROME; RESTRICTIVE CARDIOMYOPATHY, and neuropathies.
    NLM Medical Subject Headings
    U.S. National Library of Medicine, 2025
  • 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...
    NIH Genetic and Rare Diseases
    National Center for Advancing Translational Sciences
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This content should not be used in place of medically-reviewed decision support reference material or professional medical advice. Some terms may have alternate or updated definitions not reflected in this set. The definitions on this page should not be considered complete or up to date.

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