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Plasma Cell Myeloma and Associated Amino Acid Disorder: Case With Crystalline Deposition in the Cornea and Lens

Plasma Cell Myeloma and Associated Amino Acid Disorder: Case With Crystalline Deposition in the... Abstract PLASMA cell myeloma is at present a commonly recognized disease which is believed to represent autonomous neoplasia arising from those cell populations which are normally responsible for the synthesis of circulating immunoglobulins.1 The finding of marked proliferation of myeloma cells in the bone marrow is no longer a prerequisite for establishing the diagnosis because of more refined techniques available for study of dysproteinemic states. The γ-globulin molecule has, as its major polypeptide subunits, light (L) and heavy (H) chains, interconnected by disulfide bonds.2 It is conceivable that γ-globulin dysynthesis can occur at the level of the basic unit of a polypeptide, the amino acid. A case of what is believed to be a variant of multiple myeloma is reported, having as unique features amino-acidemia with a secondary amino-aciduria of the overflow type and visible deposition of crystalline material in the cornea and lens resulting in impairment of vision. References 1. Osserman, E.F., and Takatsuki, K.: Plasma Cell Myeloma: Gamma Globulin Synthesis and Structure: A Review of Biochemical and Clinical Data With the Description of a Newly Recognized and Related Syndrome "Hγ-2 Chain (Franklin's) Disease," Medicine 42:357, 1963.Crossref 2. Merler, E., and Rosen, F.S.: The Gamma Globulins: I. The Structure and Synthesis of the Immunoglobulins , New Eng J Med 275:480, 1966.Crossref 3. Stein, W.H.; Bearn, A.G.; and Moore, S.: The Amino Acid Content of the Blood and Urine in Wilson's Disease , J Clin Invest 33:410, 1954.Crossref 4. Evered, D.F.: The Excretion of Amino Acids by the Human: A Quantitative Study With Ion-Exchange Chromatography , Biochem J 62:416, 1956. 5. Engle, R.L., Jr., and Wallis, L.A.: Multiple Myeloma and the Adult Fanconi Syndrome , Amer J Med 22:5, 1957.Crossref 6. Sirota, J.H., and Hamerman, D.: Renal Function Studies in an Adult Subject With Fanconi Syndrome , Amer J Med 16:138, 1954.Crossref 7. Osserman, E.F.: Plasma Cell Myeloma: II. Clinical Aspects , New Eng J Med 261:1006, 1959.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Plasma Cell Myeloma and Associated Amino Acid Disorder: Case With Crystalline Deposition in the Cornea and Lens

Archives of Internal Medicine , Volume 120 (3) – Sep 1, 1967

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Publisher
American Medical Association
Copyright
Copyright © 1967 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1967.00300030095019
Publisher site
See Article on Publisher Site

Abstract

Abstract PLASMA cell myeloma is at present a commonly recognized disease which is believed to represent autonomous neoplasia arising from those cell populations which are normally responsible for the synthesis of circulating immunoglobulins.1 The finding of marked proliferation of myeloma cells in the bone marrow is no longer a prerequisite for establishing the diagnosis because of more refined techniques available for study of dysproteinemic states. The γ-globulin molecule has, as its major polypeptide subunits, light (L) and heavy (H) chains, interconnected by disulfide bonds.2 It is conceivable that γ-globulin dysynthesis can occur at the level of the basic unit of a polypeptide, the amino acid. A case of what is believed to be a variant of multiple myeloma is reported, having as unique features amino-acidemia with a secondary amino-aciduria of the overflow type and visible deposition of crystalline material in the cornea and lens resulting in impairment of vision. References 1. Osserman, E.F., and Takatsuki, K.: Plasma Cell Myeloma: Gamma Globulin Synthesis and Structure: A Review of Biochemical and Clinical Data With the Description of a Newly Recognized and Related Syndrome "Hγ-2 Chain (Franklin's) Disease," Medicine 42:357, 1963.Crossref 2. Merler, E., and Rosen, F.S.: The Gamma Globulins: I. The Structure and Synthesis of the Immunoglobulins , New Eng J Med 275:480, 1966.Crossref 3. Stein, W.H.; Bearn, A.G.; and Moore, S.: The Amino Acid Content of the Blood and Urine in Wilson's Disease , J Clin Invest 33:410, 1954.Crossref 4. Evered, D.F.: The Excretion of Amino Acids by the Human: A Quantitative Study With Ion-Exchange Chromatography , Biochem J 62:416, 1956. 5. Engle, R.L., Jr., and Wallis, L.A.: Multiple Myeloma and the Adult Fanconi Syndrome , Amer J Med 22:5, 1957.Crossref 6. Sirota, J.H., and Hamerman, D.: Renal Function Studies in an Adult Subject With Fanconi Syndrome , Amer J Med 16:138, 1954.Crossref 7. Osserman, E.F.: Plasma Cell Myeloma: II. Clinical Aspects , New Eng J Med 261:1006, 1959.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Sep 1, 1967

References