Primary Amyloidosis

Primary Amyloidosis

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Write a short note on AL amyloid.
Answer:

Primary amyloidosis consists of AL fibril protein which is derived from the lambda chain and more common from the kappa chain. Primary amyloidosis is more common in developed countries.

Associated disease: Plasma cell dyscrasias, For Example. multiple myeloma, B-cell lymphoma, heavy chain disease, solitary plasmacytoma.

Primary amyloidosis Pathogenesis

• The stimulus for the production of AL amyloid is some disorder of immunoglobulin synthesis, For Example. multiple myeloma and B cell lymphoma.
• Excessive immunoglobulin production is present in the form of monoclonal gammopathy. This takes place by the monoclonal proliferation of plasma cells, B lymphocytes, and their precursors.
• There is partial degradation in the form of limited proteolysis of larger protein molecules in macrophages which are associated with AL protein.
• Non-fibrillar components like AP and glycosaminoglycans play some role in the folding and aggregation of viral proteins which are insoluble.
• Organ distribution: Kidney, heart, bowel, nerve, skin, respiratory tract, and skeletal muscles.

Primary amyloidosis

Investigations for Primary Amyloidosis

• Serum/urine electrophoresis
• Immunoelectrophoresis
• Bone marrow aspiration
• Specific immunoassays with anti-kappa antibodies.

primary amyloidosis Treatment

Treatment for primary amyloidosis is targeted at reducing the underlying clonal expansion of plasma cells.