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07-SEPTEMBER-2008 03:17:44 - AL amyloidosis AL amyloidosis Classification and external resources ICD-10 E85. ICD-9 277.3 OMIM 254500 DiseasesDB 315 eMedicine med/3363 AL amyloidosis, or primary amyloidosis, is the most common form of systemic amyloidosis in the US.1 It occurs in 5 to 15% of people with multiple myeloma.citation needed The AL refers to amyloid light chain.2 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.3 A combination of bortezomib and dexamethasone has been proposed,4 as has melphalan and dexamethasone.5 References ^ Gertz MA June 2004. The classification and typing of amyloid deposits. Am. J. Clin. Pathol. 121 6: 787-9. doi:10.1309/TR4L-GLVR-JKAM-V5QT. PMID 15198347. ^ Amyloidosis Causes, Diagnosis, Symptoms, and Treatment on MedicineNet.com. ^ BU. ^ Kastritis E, Anagnostopoulos A, Roussou M, et al October 2007. Treatment of light chain AL amyloidosis with the combination of bortezomib and dexamethasone. Haematologica 92 10: 1351-8. doi:10.3324/haematol.11325. PMID 18024372. ^ Palladini G, Perfetti V, Obici L, et al April 2004. 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. Blood 103 8: 2936-8. doi:10.1182/blood-2003-08-2788. PMID 15070667. v d e Metabolic disease: amyloidosis E85, 277.3 Forms of amyloid Serum amyloid P component - Serum amyloid A - Transthyretin - Beta-2 microglobulin - Bence Jones protein/Multiple myeloma - Amyloid precursor protein/Beta amyloid - Amylin Systemic amyloidosis AL amyloidosis - AA amyloidosis - Haemodialysis-associated amyloidosis - Senile systemic amyloidosis - Finnish type amyloidosis - Familial Merranean fever Organ-limited amyloidosis heart: Cardiac amyloidosis brain: Familial amyloid neuropathy - Cerebral amyloid angiopathy - Alzheimer's disease kidney: Familial renal amyloidosis cutaneous: Primary cutaneous amyloidosis Retrieved from http://en..org/wiki/AL_amyloidosis Categories: Metabolic disorders | DiseasesHidden categories: All articles with statements | Articles with statements since June 2008 Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page This page was last modified on 13 June 2008, at 18:05
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