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Resolution of Factor X Deficiency in Primary Amyloidosis Following Splenectomy

Resolution of Factor X Deficiency in Primary Amyloidosis Following Splenectomy Abstract • A 57-year-old man with primary amyloidosis was initially seen with hematuria, cutaneous bleeding, and hepatosplenomegaly. Factor X was determined to be 10% to 16% of normal plasma values. Administration of vitamin K-dependent factor concentrate transiently improved in vitro clotting tests but did not alter the clinical course. Following a splenectomy, bleeding ceased and factor X levels returned to normal, remaining so despite discontinuation of factor concentrate infusion. Amyloid fibrils extracted from the patient's spleen were determined to be derived from λV1 light chains. The importance of splenectomy as an effective therapeutic modality is discussed. (Arch Intern Med 1983;143:597-599) References 1. Greipp PR, Kyle RA, Bowie EJW: Factor X deficiency in amyloidosis: A critical review. Am J Hematol 1981;11:443-450.Crossref 2. Zeitler KD, Blatt PM: Amyloidosis and factor X deficiency. South Med J 1982;75:306-308, 312.Crossref 3. Kyle RA, Bayrd ED: Amyloidosis: A review of 236 cases. Medicine 1975;54:271-299.Crossref 4. Furie B, Greene E, Furie BC: Syndrome of acquired factor X deficiency and systemic amyloidosis: In vivo studies of the metabolic fate of factor X. N Engl J Med 1977;297:81-85.Crossref 5. Cohen DH, Pras M, Franklin EC, et al: Characterization of amyloid proteins from amyloidosis presenting with factor X deficiency, abstracted. Clin Res 1982;30:344A. 6. Korsan-Bengsten K, Hjort PF, Ygge J: Acquired factor X deficiency in a patient with amyloidosis. Thromb Haemost 1962;7:558-566. 7. Menache D, Boivin P: Deficit acquis en facteur X chez un malad atteint d'amyloise primitive: Injection d'une fraction. Nouv Rev Fr Hematol 1962;2:868-887. 8. Howell M: Acquired factor X deficiency associated with systematized amyloidosis: A report of a case. Blood 1963;21:739-744. 9. Furie B, Voo L, McAdam KP, et al: Mechanism of factor X deficiency in systemic amyloidosis. N Engl J Med 1981;304:827-830.Crossref 10. McPherson RA, Onstad JW, Ugoretz RJ, et al: Coagulopathy in amyloidosis: Combined deficiency of factor IX and X. Am J Hematol 1977;3:225-235.Crossref 11. Blatt PM, Lundblad RL, Kingdon HS, et al: Thrombogenic materials in prothrombin complex concentrates. Ann Intern Med 1974;81:766-770.Crossref 12. Greipp PR, Kyle RA, Bowie EJW: Factor X deficiency in primary amyloidosis: Resolution after splenectomy. N Engl J Med 1979;301:1050-1051.Crossref 13. Solomon A, Frangione B, Franklin EC: Bence Jones proteins and light chains of immunoglobulins. J Clin Invest 1982;70:453-460.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Resolution of Factor X Deficiency in Primary Amyloidosis Following Splenectomy

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Publisher
American Medical Association
Copyright
Copyright © 1983 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1983.00350030211041
Publisher site
See Article on Publisher Site

Abstract

Abstract • A 57-year-old man with primary amyloidosis was initially seen with hematuria, cutaneous bleeding, and hepatosplenomegaly. Factor X was determined to be 10% to 16% of normal plasma values. Administration of vitamin K-dependent factor concentrate transiently improved in vitro clotting tests but did not alter the clinical course. Following a splenectomy, bleeding ceased and factor X levels returned to normal, remaining so despite discontinuation of factor concentrate infusion. Amyloid fibrils extracted from the patient's spleen were determined to be derived from λV1 light chains. The importance of splenectomy as an effective therapeutic modality is discussed. (Arch Intern Med 1983;143:597-599) References 1. Greipp PR, Kyle RA, Bowie EJW: Factor X deficiency in amyloidosis: A critical review. Am J Hematol 1981;11:443-450.Crossref 2. Zeitler KD, Blatt PM: Amyloidosis and factor X deficiency. South Med J 1982;75:306-308, 312.Crossref 3. Kyle RA, Bayrd ED: Amyloidosis: A review of 236 cases. Medicine 1975;54:271-299.Crossref 4. Furie B, Greene E, Furie BC: Syndrome of acquired factor X deficiency and systemic amyloidosis: In vivo studies of the metabolic fate of factor X. N Engl J Med 1977;297:81-85.Crossref 5. Cohen DH, Pras M, Franklin EC, et al: Characterization of amyloid proteins from amyloidosis presenting with factor X deficiency, abstracted. Clin Res 1982;30:344A. 6. Korsan-Bengsten K, Hjort PF, Ygge J: Acquired factor X deficiency in a patient with amyloidosis. Thromb Haemost 1962;7:558-566. 7. Menache D, Boivin P: Deficit acquis en facteur X chez un malad atteint d'amyloise primitive: Injection d'une fraction. Nouv Rev Fr Hematol 1962;2:868-887. 8. Howell M: Acquired factor X deficiency associated with systematized amyloidosis: A report of a case. Blood 1963;21:739-744. 9. Furie B, Voo L, McAdam KP, et al: Mechanism of factor X deficiency in systemic amyloidosis. N Engl J Med 1981;304:827-830.Crossref 10. McPherson RA, Onstad JW, Ugoretz RJ, et al: Coagulopathy in amyloidosis: Combined deficiency of factor IX and X. Am J Hematol 1977;3:225-235.Crossref 11. Blatt PM, Lundblad RL, Kingdon HS, et al: Thrombogenic materials in prothrombin complex concentrates. Ann Intern Med 1974;81:766-770.Crossref 12. Greipp PR, Kyle RA, Bowie EJW: Factor X deficiency in primary amyloidosis: Resolution after splenectomy. N Engl J Med 1979;301:1050-1051.Crossref 13. Solomon A, Frangione B, Franklin EC: Bence Jones proteins and light chains of immunoglobulins. J Clin Invest 1982;70:453-460.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Mar 1, 1983

References