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Lymphoma - B cell neoplasms

Plasma cell neoplasms

Primary amyloidosis


Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 16 March 2011, last major update March 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● Monoclonal proliferation of plasma cells secreting free light chains (usually lambda), which are deposited as amyloid
Amyloid: fibrillary protein, 95% composed of non-branching fibrils 7.5 to 10 nm thick with variable length in a beta-pleated sheet conformation; 5% is serum amyloid P component, made of proteoglycans and highly sulfated glycosaminoglycans
● Amyloid is deposited in various tissues causing local damage

Terminology
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● Due to primary amyloidosis (AL type), secondary amyloidosis (AA type) or familial amyloidosis (AF type)

Clinical features
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● Only primary amyloidosis is associated with plasma cell dyscrasias
● Causes include overt multiple myeloma (20%), plasmacytomas of bone marrow or minor population of plasma cells
● Rare (2,000 cases/year in US), usually ages 60-70 years, 2/3 are men
Symptoms: weight loss, fatigue, heart failure, peripheral neuropathy, nephrotic syndrome (1/3), bleeding (acquired factor X deficiency due to its binding to amyloid protein)
● 90% have serum or urine monoclonal protein, although electrophoretic spike may be small; detect with serum/urine immunofixation or serum free light chain ratio analysis if amyloidosis suspected
● Biopsy is 80% sensitive in rectum and abdominal wall fat, 50% in bone marrow (may be confined to blood vessel walls only)
● Median survival 1-2 years; death due to amyloid-related cardiac disease, renal failure, infection, hemorrhage
● Patients with plasma cell myeloma and amyloidosis have shorter survival thatn amyloidosis alone

Case reports
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● 12 year old girl with amyloidosis and extramedullary plasmacytoma in larynx (Hum Pathol 2001; 32:132)
● Subcutaneous nodular amyloidosis due to lambda light chains (Hum Pathol 2001;32:346)
● Monoclonal immunoglobulin deposition disease associated with nephrotic syndrome and subsequent heavy chain disease with amyloidosis (Am J Surg Pathol 2003;27:1477)

Treatment
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● Alkylating agent chemotherapy

Micro description
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● Pale pink amorphous material, apple-green birefringence with Congo red stain and polarized light microscopy
● Check vessel walls in bone marrow biopsies for amyloid deposition

Electron microscopy description
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● Fine, linear, nonbranching fibrils

End of Lymphoma - B cell neoplasms > Plasma cell neoplasms > Primary amyloidosis


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