LETTER
Unusual Involvement of Bone in Primary Systemic Amyloidosis
Garcia-Gasco Pilar, MD;
Morata Aldea Carmen, MD; and
Perello Rosso Alfonso, MD
15 August 1998 | Volume 129 Issue 4 | Pages 338-339
TO THE EDITOR:
Primary systemic amyloidosis is characterized by extracellular deposits of type AL amyloid protein in the absence of multiple myeloma. Many organs are affected in this disease, but involvement of bone is rare [1-4]. We report a case of primary systemic amyloidosis and multiple bone lesions produced by amyloid protein deposits with an osteoblastic appearance.
A 69-year-old man was admitted for the evaluation of a nephrotic syndrome. Analyses revealed an acquired deficit of factor X, measures of dissociated cholestasis, and the presence of a 
type IgA monoclonal gammopathy (1123 mg/100 mL) in immunoelectrophoresed plasma. Computed tomography showed multiple blastic lesions in all vertebral bodies, the sacrum, and the ilium; bone gammography showed an abnormal uptake in the same sites. A bone marrow biopsy specimen was normal, but a biopsy sample taken from one of the iliac lesions disclosed the presence of type AL amyloid protein and excluded the existence of neoplastic cells. A renal biopsy revealed similar deposits. Echocardiography was suggestive of cardiac impairment due to amyloidosis. The progressive increases in 
-glutamyltransferase and alkaline phosphatase levels likewise suggested liver involvement. Combined treatment with melphalan and prednisone was started but had to be discontinued because of the development of bone marrow toxicity. The patient sustained crushing of several vertebrae, and renal function gradually worsened. He died after 2 years of follow-up.
In type AL systemic amyloidosis not associated with myeloma, bone involvement is exceptional. In all cases reported in the literature, patients with amyloid infiltration of bone presented with generalized demineralization, destructive bone lesions, or pathologic fractures usually located in the femoral neck or vertebrae. The major skeletal involvement observed in our patient and the fact that this involvement manifested radiologically as sclerotic bone lesions distinguish our case from those described previously. These findings also suggest that this abnormality should be considered in the differential diagnosis of osteoblastic bone lesions.
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Author and Article Information
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Hospital General Universitario "La Fe"; Valencia, Spain
1. Subbarao K, Jacobson HG. Amyloidosis and plasma cell dyscrasias of the musculoskeletal system. Semin Roentgenol. 1986; 21:139-49.
2. Axelsson U, Hallen A, Rausing A. Amyloidosis of bone. J Bone Joint Surg (Br). 1970; 52:717-23.
3. Khojasteh A, Lowry K, Arnold LK, Farhangi M. Bone lesions in primary amyloidosis. Am J Hematol. 1979; 7:77-86.
4. Kramer MR, van Dijk JM, Hadas I, Hershko C. Destructive bone lesions in primary amyloidosis. Postgrad Med J. 1986; 62:1037-41.
5. Glenner GG. Amyloid deposits and amyloidosis. N Engl J Med. 1980; 302:1333-43.
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