9769/1 Immunoglobulin deposition disease


Synonyms

Systemic light chain disease
Primary amyloidosis

Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77

/span>rimary 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 ?-pleated sheet structure (AL amyloid) that binds Congo red dye with characteristic birefringence 1
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Gertz MA, Kyle RA (2003)
Amyloidosis with IgM monoclonal gammopathies.
Semin Oncol 30: 325-8



2
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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



3
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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



4
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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



5
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Kyle RA, Gertz MA (1995)
Primary systemic amyloidosis: clinical and laboratory features in 474 cases.
Semin Hematol 32: 45-59



6
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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 macro?phages 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
7
Click to access Pubmed
Kyle RA, Gertz MA (1995)
Primary systemic amyloidosis: clinical and laboratory features in 474 cases.
Semin Hematol 32: 45-59



8
Click to access Pubmed
Serpell LC, Sunde M, Blake CC (1997)
The molecular basis of amyloidosis.
Cell Mol Life Sci 53: 871-87



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