Amyloidosis: a changing clinical perspective.
Primary amyloidosis is a plasma cell dyscrasia characterised by excess production of abnormal immunoglobulin light chains with their subsequent accumulation in kidneys, heart, liver as well as gastrointestinal tract and bone marrow 1-21, 2. These tissue deposits take the form of a fibrillar protein which damages the involved organ in proportion to the extent of the infiltration and roughly parallels the duration of the disease. Most cases have evidence of the underlying lymphoplasmacytoid neoplasm recognisable in two ways. Firstly, the monoclone appears in the serum [2]. Secondly is a morphologically and immunohistochemically distinctive cellular infiltrate in the bone marrow [3] that has a specific microscopic and ultrastructural pattern 4-54, 5. Interestingly occasional patients, who survive long enough, may progress to multiple myeloma [6] but the correlation is variable [7].
Jacobs
Peter
P
College of Medicine - University of Nebraska Medical School, Nebraska, USA.
Ruff
Paul
P
Wood
Lucille
L
Moodley
Devan
D
Mansvelt
Erna
E
D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000473:pathology / Q000628:therapy*; D000911:Antibodies, Monoclonal / Q000097:blood; D001853:Bone Marrow / Q000473:pathology; D016026:Bone Marrow Transplantation; D003078:Colchicine / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D003937:Diagnosis, Differential; D019468:Disease Management; D005260:Female; D006801:Humans; D007167:Immunotherapy; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000473:pathology; D008297:Male; D008558:Melphalan / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D008775:Methylprednisolone / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D008875:Middle Aged; D010950:Plasma Cells / Q000473:pathology; D011241:Prednisone / Q000009:adverse effects / Q000627:therapeutic use; D016499:Radioimmunotherapy; D013019:South Africa / Q000453:epidemiology; D015996:Survival Rate
D000911:Antibodies, Monoclonal; D008558:Melphalan; D003078:Colchicine; D011241:Prednisone; D008775:Methylprednisolone