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14-September-2008 18:38:50 - 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 - Amyloid purpura v d e Hematological malignancy/leukemia histology ICD-O 9590-9989, C81-C96, 200-208 Lymphoid/ Lymphoproliferative Lymphomas/ Lymphoid leukemias 9590-9739, 9800-9839 B cell lymphoma, leukemia most CD19, CD20 TdT+ ALL Precursor B acute lymphoblastic leukemia/lymphoma CD5+ SLL/CLL - mantle zone Mantle cell CD22+ Prolymphocytic - Hairy cell leukemia CD79a+ marginal zone Splenic marginal zone, MALT, Nodal marginal zone germinal center Follicular, Burkitt's, Diffuse large Other B Primary effusion - Lymphomatoid granulomatosis Plasma cells Plasmacytoma - Multiple myeloma - Waldenström macroglobulinemia - Heavy chain disease - MGUS - Primary amyloidosis -- Plasma cell leukemia T cell/NK cell lymphoma, leukemia most CD3, CD4, CD8 TdT+ ALL Precursor T acute lymphoblastic leukemia/lymphoma TdT- Cutaneous - Mycosis fungoides/Sézary's disease, Angioimmunoblastic - Anaplastic large cell - Hepatosplenic - Lymphomatoid papulosis Adult - Large granular lymphocyte - Prolymphocytic - Acute lymphoblastic Aggressive NK-cell leukemia Other Hodgkin's lymphoma usually B cell - Post-transplant lymphoproliferative disorder - Acute biphenotypic leukaemia Myeloid Myeloid leukemias 9840-9939 AML: Acute myeloblastic leukemia M0, M1, M2 - APL/M3 - monocyte M4, AMoL/M5, Myeloid dendritic cell leukemia - Erythroleukemia/M6 - AMKL/M7 - megakaryoblast - granulocyte Acute basophilic, Acute eosinophilic - Acute panmyelosis with myelofibrosis CML: CMoL, Philadelphia chromosome - Myeloid sarcoma Myelodysplastic- myeloproliferative 9945-9946 Juvenile myelomonocytic leukemia - Chronic myelomonocytic leukemia Myeloproliferative 9950-9964 Polycythemia vera - Essential thrombocytosis - Myelofibrosis - granulocyte CNL, Chronic eosinophilic leukemia/Hypereosinophilic syndrome Myelodysplastic 9980-9989 Sideroblastic anemia - Paroxysmal nocturnal hemoglobinuria Mast cell tumor 9740-9749 Mast cell leukemia - Mast cell sarcoma - Systemic mastocytosis Malignant histiocytosis 9750-9759 Langerhans cell histiocytosis See also hematology and immune disorders 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 9 September 2008, at 19:23
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