Primary systemic amyloidosis presenting at an early age: A case report

Abstract

International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties.Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations.IJCRI publishes Review Articles, Case Series, Case Reports, Case in Images, Clinical Images and Letters to Editor. Website: www.ijcasereportsandimages.comPrimary systemic amyloidosis presenting at an early age:A case report Arghya Chattopadhyay, Shatarupa Dutta, Amitava Majumder, Jayanta Das ABSTRACT Introduction: Primary systemic amyloidosis arises from clonal B cell disorder and may be associated with myeloma or lymphoma. Amyloid deposition can occur in any organ in the body as well as skin lesions. Both sexes are affected by this disease, with onset most commonly in the sixth decade or after. Case Report: A 26-year-old female was presented with gradually changing quality of voice, diffuse pigmentation all over body and lymphadenopathy in upper cervical group. Biopsy of a lymph node showed follicular hyperplasia with a few discrete large cells. Over a period of time, she developed purpuric rash over extremities and periorbital area, respiratory distress and heart failure. Echocardiography revealed cardiac amyloidosis and rectal biopsy with Congo red staining was positive for amyloidosis. Skin biopsy from hyperpigmented lesion of right forearm was also suggestive of amyloidosis and bone marrow examination showed plasma cell dyscrasia with a small M-band in serum protein electrophoresis. Finally, it was diagnosed a case of primary systemic amyloidosis. Conclusion:We report a primary systemic amyloidosis with an early age presentation.

Primary systemic amyloidosis with unusual dermatological manifestations: A rare case report

“…Among 25 different proteins identified to produce amyloid fibrils, most are constituents of plasma. [ 4 ]…”