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Q J Med 2000; 93: 257-260
© 2000 Association of Physicians

Editorial

Prospects for effective treatment of AL amyloidosis?

N. Iggo, T. Littlewood and C.G. Winearls

The Trafford Department of Renal Medicine, Royal Sussex County Hospital, Brighton BN2 5BE Department of Haematology, Oxford Radcliffe Hospital, Oxford Oxford Kidney Unit, The Churchill, Oxford Radcliffe Hospital, Oxford

Systemic amyloidosis is a condition characterized by the extracellular deposition of insoluble fibrillar proteins, leading to organ dysfunction. Its various forms are classified according to the nature of the precursor protein constituent of the fibril for example: immunoglobulin light chains in AL amyloidosis, heavy chains in AH amyloid, serum amyloid A protein, (a component of the acute-phase response) in AA amyloid, and a heterogeneous group of abnormal proteins in the familial amyloidoses (reviewed in reference 1) (Figure 1Go). AL amyloidosis is at present considered an incurable disease, with 8 months median survival from the time of diagnosis. The prognosis depends on the dominant organ system affected: patients with cardiac involvement have a median survival of 6 months, compared to 21 months in those with renal disease.



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  		Figure 1. Renal biopsy of a patient with AL amyloid. Glomerulus stained with PAS (Periodic Acid Schiff) showing deposition of amorphous material. Congo red staining . . . [Full Text of this Article]

 
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