Primary Systemic Amyloidosis. a Case Report.

Primary systemic amyloidosis is a rare entity. We report a case of primary systemic primary amyloidosis, an elderly male presented with generalized weakness since 6 months. Clinical examination revealed typical waxy lesions in the periorbital area with macroglossia. Serum electrophoresis was normal, however, bone marrow examination showed increased plasma cell with a good number of both binucleated and immature plasma cells. Diagnosis was confirmed by skin biopsy using haemotoxylin & eosin staining and congo red staining. Polarised microscopy was not done because of unavailability. We present the myriad manifestations of this uncommon disease entity. Case Report *Corresponding author: Dr Tanima Dwivedi, Post Graduate, Department of Pathology, KLE University’s J.N. Medical College, Belgaum, Karnataka(India)-590010 Phone: +91 7406741788, 9818774359 E-mail: [email protected] A-42 Primary Systemic Amyloidosis Annals of Pathology and Laboratory Medicine, Vol. 03, No. 01, January March 2016 Introduction Primary systemic amyloidosis (AL amyloidosis) is a rare disease with an age-adjusted incidence of 5.1 to 12.8 per million person-years.[1] It is a plasma cell dyscrasia of unknown etiology with production of monoclonal chains.[2] Immunoglobulin light chains, or fragments of light chains, produced by these plasma cell clones form extracellular amyloid fibrils which are deposited in the organs.[3] Most frequently organ involved are heart, smooth and skeletal muscle, and other soft tissues, kidney, liver and spleen.[2] It’s the disease of elderly.