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Pathology - Primary amyloidosis
Definition: A plasma cell neoplasm characterized by the accumulation of AL amyloid in several tissues.
Epidemiology • Uncommon ailment. The median age at diagnosis is 65 years, with a predominance of males.
Aetiology The majority of patients possess an underlying plasma cell neoplasm yet do not fulfill the diagnostic criteria for plasma cell myeloma. Pathogenesis • AL amyloid consists of immunoglobulin light chains produced by monoclonal plasma cells, which aggregate in diverse tissues in a β-pleated sheet configuration. • The deposited amyloid comprises both intact light chains and fragments of the variable NH2-terminal region.
Presentation • Clinical manifestations associated with amyloid accumulation in several organs. Commonly affected areas encompass the skin, kidneys, heart, liver, intestines, and peripheral nerves. Characteristic manifestations include purpura, peripheral neuropathy, cardiac insufficiency, nephrotic syndrome, and malabsorption.
Histopathology • Amyloid can be identified in several tissues as a pink, amorphous material. The Congo Amyloid exhibits red staining under ordinary light microscopy and 'apple green' under polarized light. Bone marrow biopsies generally reveal a little elevation in plasma cells, which may present as either normal or abnormal. The plasma cells exhibit monotypism for either kappa or lambda light chains.
Prognosis: • Dismal prognosis with a median survival of merely 2 years from diagnosis. The predominant cause of mortality is amyloid-related heart failure.
Definition: A plasma cell neoplasm characterized by the accumulation of AL amyloid in several tissues.
Epidemiology • Uncommon ailment. The median age at diagnosis is 65 years, with a predominance of males.
Aetiology The majority of patients possess an underlying plasma cell neoplasm yet do not fulfill the diagnostic criteria for plasma cell myeloma. Pathogenesis • AL amyloid consists of immunoglobulin light chains produced by monoclonal plasma cells, which aggregate in diverse tissues in a β-pleated sheet configuration. • The deposited amyloid comprises both intact light chains and fragments of the variable NH2-terminal region.
Presentation • Clinical manifestations associated with amyloid accumulation in several organs. Commonly affected areas encompass the skin, kidneys, heart, liver, intestines, and peripheral nerves. Characteristic manifestations include purpura, peripheral neuropathy, cardiac insufficiency, nephrotic syndrome, and malabsorption.
Histopathology • Amyloid can be identified in several tissues as a pink, amorphous material. The Congo Amyloid exhibits red staining under ordinary light microscopy and 'apple green' under polarized light. Bone marrow biopsies generally reveal a little elevation in plasma cells, which may present as either normal or abnormal. The plasma cells exhibit monotypism for either kappa or lambda light chains.
Prognosis: • Dismal prognosis with a median survival of merely 2 years from diagnosis. The predominant cause of mortality is amyloid-related heart failure.
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