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WHO Classification of Tumours
Primary amyloidosis
Tumours of haematopoietic and lymphoid tissues


Definition

Primary amyloidosis is caused by a plasma cell, or rarely, a lymphoplasmacytic neoplasm that secretes intact or fragments of abnormal immunoglobulin light chains, or rarely, heavy chains, which deposit in various tissues and form a ß-pleated sheet structure (AL amyloid) that binds Congo red dye with characteristic birefringence
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Gertz MA, Kyle RA (2003)
Amyloidosis with IgM monoclonal gammopathies.
Semin Oncol 30: 325-8




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Gertz MA, Kyle RA, Noel P (1993)
Primary systemic amyloidosis: a rare complication of immunoglobulin M monoclonal gammopathies and Waldenström's macroglobulinemia.
J Clin Oncol 11: 914-20




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Gono T, Yazaki M, Fushimi T, Suzuki T, Uehara T, Sano K, Kametani F, Ito N, Matsushita M, Nakamura S, Hoshii Y, Matsuda M, Ikeda S (2006)
AH amyloidosis associated with lymphoplasmacytic lymphoma secreting a monoclonal gamma heavy chain carrying an unusual truncated D segment.
Am J Kidney Dis 47: 908-14




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Kjaeraas S, Husby G, Sletten K (2006)
The amino acid sequence of an AL-protein, AL-KH, isolated from the heart of a patient with Waldenstroms macroglobulinemia and amyloidosis.
Amyloid 13: 260-2




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Kyle RA, Gertz MA (1995)
Primary systemic amyloidosis: clinical and laboratory features in 474 cases.
Semin Hematol 32: 45-59




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Serpell LC, Sunde M, Blake CC (1997)
The molecular basis of amyloidosis.
Cell Mol Life Sci 53: 871-87



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AL amyloid is composed of intact immunoglobulin light chains or rarely, heavy chains that are secreted by monoclonal plasma cells and then ingested, processed and discharged by macro­phages into the extracellular matrix. The accumulated amyloid includes both intact light chain and fragments of the variable (V) NH2-terminus region. All light chain V region fragments are potentially amyloidogenic, and all plasma cell neoplasms that produce VλVI have AL

Click to access Pubmed
Kyle RA, Gertz MA (1995)
Primary systemic amyloidosis: clinical and laboratory features in 474 cases.
Semin Hematol 32: 45-59




Click to access Pubmed
Serpell LC, Sunde M, Blake CC (1997)
The molecular basis of amyloidosis.
Cell Mol Life Sci 53: 871-87



.

> Related Topics
Immunoglobulin deposition diseases
Monoclonal light and heavy chain deposition diseases
Introduction: Classification of lymphoid neoplasms