AL amyloidosis

AL amyloidosis

AL amyloidosis, or primary amyloidosis, is the most common form of systemic amyloidosis in the US.cite journal |author=Gertz MA |title=The classification and typing of amyloid deposits |journal=Am. J. Clin. Pathol. |volume=121 |issue=6 |pages=787–9 |year=2004 |month=June |pmid=15198347 |doi=10.1309/TR4L-GLVR-JKAM-V5QT |url=http://ajcp.metapress.com/openurl.asp?genre=article&issn=0002-9173&volume=121&issue=6&spage=787]

It occurs in 5 to 15% of people with multiple myeloma.Fact|date=June 2008

The "AL" refers to amyloid light chain.cite web |url=http://www.medicinenet.com/amyloidosis/article.htm |title=Amyloidosis Causes, Diagnosis, Symptoms, and Treatment on MedicineNet.com |format= |work= |accessdate=]

Presentation

The Bence Jones proteins produced are necessary to produce amyloidosis but other factors are required for amyloidosis to occur. Most people with AL amyloidosis do not have multiple myeloma, or any other B cell tumour. They often have a serum gammopathy, and it is unknown whether these cases will ultimately evolve into myeloma, as these people often die from their amyloidosis before malignant transformation occurs.

Treatment

Treatment can involve application of chemotherapy similar to that used in multiple myeloma.cite web |url=http://www.bu.edu/amyloid/doctors/features/clinical-al.html |title=BU | Amyloid Treatment & Research Program | Doctors | Clinical Features | AL Amyloidosis |format= |work= |accessdate=]

A combination of bortezomib and dexamethasone has been proposed,cite journal |author=Kastritis E, Anagnostopoulos A, Roussou M, "et al" |title=Treatment of light chain (AL) amyloidosis with the combination of bortezomib and dexamethasone |journal=Haematologica |volume=92 |issue=10 |pages=1351–8 |year=2007 |month=October |pmid=18024372 |doi=10.3324/haematol.11325 |url=http://www.haematologica.org/cgi/pmidlookup?view=long&pmid=18024372] as has melphalan and dexamethasone.cite journal |author=Palladini G, Perfetti V, Obici L, "et al" |title=Association of melphalan and high-dose dexamethasone is effective and well tolerated in patients with AL (primary) amyloidosis who are ineligible for stem cell transplantation |journal=Blood |volume=103 |issue=8 |pages=2936–8 |year=2004 |month=April |pmid=15070667 |doi=10.1182/blood-2003-08-2788 |url=http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=15070667]

References


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