Primary Amyloidosis Disease Description

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Amyloidosis is a disease characterized by the extracellular accumulation of fibrils in different organs in the body. The precursor proteins may vary but all contain an antiparallel β-pleated sheet configuration thus resulting to their amyloidogenic properties and unique reaction (the production of a green birefringence under polarized light) when stained with Congo red. All types of amyloid are comprised of a serum amyloid P component, a 25-kD glycoprotein, and a member of the C-reactive-protein-containing pentraxin family. Amyloidosis may be classified into diverse groups of local and systemic diseases with primary amyloidosis as the most widely occurring.

In primary amyloidosis, the fibrils that build up in various organs are made up of the N-terminal acid residues of the variable section of an immunoglobin light chain; hence the other name primary amyloidosis is known for: immunoglobin light chain amyloidosis. Here, the clonal plasma cells predominantly express λ light chains over κ light chains and an increased incidence of monoclonal λ type VI can be observed. There are various symptoms that could hint people of primary amyloidosis but in order to be confirmed, a biopsy documentation of the amyloid is required.

The typical number of cases of primary amyloidosis reaches 8 per million yearly. It normally occurs in patients over the age of 50; only about 1% are below the age of 40. And it occurs in men in about twice as much as it does in women. Patients with primary amyloidosis are typically proven to have only a median survival of 2 years or less. One reason to this may be the fact that multisystem organ involvement is quite usual in this disease, with the most commonly affected ones being the kidney, the heart, and the pe...

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... is also of note that high-dose chemotherapy with peripheral blood stem cell transportation has led to the treatment of nephrotic syndrome and biopsy-improvement of amyloid organ involvement in some isolated cases, and that it has shown very promising results. However, strict care measures must still be observed regardless of whether chemotherapy or marrow transplant is used.

Works Cited

Cotter, F., & Rund, D. (2001). Historical Review, Amyloidosis: A Convoluted Story. British Journal of Haematology. (114). Retrieved from http://www.bloodmed.com/home/hannpdf/bjh2999.pdf

Linman, J. (1975). Hematology: Physiologic, Pathophysiologic, and Clinical Principles. New York: Macmillan Publishing Co., Inc.

Hoffman, R., Benz, E., et al. (2005). Hematology: Basic Principles and Practice. Philadephia, Pennsylvannia: Elsevier Inc.

Brenner, B. (2004). The Kidney. Saunder’s.

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