Nonoperative Management of Spontaneous Splenic Rupture in a Patient with Light-Chain Amyloidosis: A Case Report

Amyloid light-chain (AL) amyloidosis is a rare plasma cell dyscrasia characterized by tissue deposition of amyloid fibrils ( 1 Falk R.H. Comenzo R.L. Skinner M. The systemic amyloidoses. N Engl J Med. 1997; 337: 898-909 Crossref PubMed Scopus (1069) Google Scholar ). These fibrils are composed of misfolded monoclonal light chains secreted by a clonal population of plasma cells, with an incidence in the United States of 5–12 per million persons per year ( 2 Bird J. Cavenagh J. Hawkins P. Lachmann H. Mehta A. Samson D. Guidelines Working Group of UK Myeloma ForumGuidelines on the diagnosis and management of AL amyloidosis. Br J Haematol. 2004; 125: 681-700 Crossref PubMed Scopus (143) Google Scholar ). Splenic involvement in AL amyloidosis is found in 5%–10% of cases ( 3 Sucker C. Hetzel G.R. Grabensee B. Stockschlaeder M. Scharf R.E. Amyloidosis and bleeding: pathophysiology, diagnosis, and therapy. Am J Kidney Dis. 2006; 47: 947-955 Abstract Full Text Full Text PDF PubMed Scopus (99) Google Scholar ), with 4%–13% of patients having splenomegaly and 24% having splenic dysfunction ( 4 Oran B. Wright D.G. Seldin D.C. McAneny D. Skinner M. Sanchorawala V. Spontaneous rupture of the spleen in AL amyloidosis. Am J Hematol. 2003; 74: 131-135 Crossref PubMed Scopus (44) Google Scholar ).