TY - JOUR
T1 - The clinical spectrum of light chain myeloma. A study of 35 patients with special reference to the occurrence of amyloidosis
AU - Stone, Marvin J.
AU - Frenkel, Eugene P.
N1 - Funding Information:
From the Evelyn L. Overton Hematology-Oncology Laboratory, Department of Internal Medicine, The University of Texas Southwestern Medical School and the Dallas Veterans Administration Hospital. Portions of this study were presented at the 53rd Annual Meeting of the American College of Physicians, Atlantic City, New Jersey, April 1972 (Ann intern Med 76: 871, 1972) and at the National Meeting of the American Federation for Clinical Research, Atlantic City, New Jersey, April 1973 (C/in Res 21:655, 1973). This study was supported by Grant ET-62 from the American Cancer Society, the Damon Runyon Memorial Fund, and the Texas Heart Association. This work was performed during the tenure of an Established In-vestigatorship of the American Heart Association (M.J.S.). Requests for reprints should be addressed to Dr. Marvin J. Stone, Department of Internal Medicine, The University of Texas Southwestern Medical School, 5323 Harry Hines Boulevard, Dallas, Texas 75235. Manuscript accepted June 4, 1974.
Funding Information:
Comment: This young woman initially appeared to have an isolated plasma cell tumor, but her subsequent course documented systemic involvement. Evidence for diffuse disease was supported by the quantitatively progressive lambda Bence Jones proteinuria.
Copyright:
Copyright 2018 Elsevier B.V., All rights reserved.
PY - 1975/5
Y1 - 1975/5
N2 - During a 40 month interval, 35 patients were seen with a plasma cell dyscrasia in which the only detectable monoclonal immunoglobulin abnormality consisted of excess production of either type kappa or type lambda light chains (Bence Jones protein). This group constituted 17.3 per cent of the total number of patients with plasma cell dyscrasias and 25.7 per cent of the patients with myeloma identified during the same period. Variable initial clinical presentation, the absence of a typical monoclonal serum spike and the unreliability of commonly used urine protein tests made recognition of the disorder difficult in many patients. Sulfosalicylic acid and p-toluene sulfonic acid proved valuable in screening for urine protein. Definition of "proteinuria" by quantitative, electrophoretic and immunochemical studies was essential for diagnosis. Bence Jones proteinemia was present in 80 per cent of the patients; panhypogammaglobulinemia and lytic bone lesions were demonstrable in more than 60 per cent. Although no specific morphologic abnormality of marrow plasma cells was evident by light microscopy, the absence of rouleau on peripheral blood smear was a helpful diagnostic clue. Although patients with lambda light chains presented with more Bence Jones proteinuria, this did not correlate with the severity of initial renal functional impairment or with survival when compared to patients with kappa light chains. No other clinical or laboratory observation differentiated the groups with kappa light chains from those with lambda light chains. Amyloid was identified in seven patients. Their course was dominated by the features of primary systemic amyloidosis instead of the usual findings of classic myeloma. Patients with amyloidosis had lower initial serum albumin levels, fewer lytic bone lesions and reduced survival compared to patients without amyloidosis.
AB - During a 40 month interval, 35 patients were seen with a plasma cell dyscrasia in which the only detectable monoclonal immunoglobulin abnormality consisted of excess production of either type kappa or type lambda light chains (Bence Jones protein). This group constituted 17.3 per cent of the total number of patients with plasma cell dyscrasias and 25.7 per cent of the patients with myeloma identified during the same period. Variable initial clinical presentation, the absence of a typical monoclonal serum spike and the unreliability of commonly used urine protein tests made recognition of the disorder difficult in many patients. Sulfosalicylic acid and p-toluene sulfonic acid proved valuable in screening for urine protein. Definition of "proteinuria" by quantitative, electrophoretic and immunochemical studies was essential for diagnosis. Bence Jones proteinemia was present in 80 per cent of the patients; panhypogammaglobulinemia and lytic bone lesions were demonstrable in more than 60 per cent. Although no specific morphologic abnormality of marrow plasma cells was evident by light microscopy, the absence of rouleau on peripheral blood smear was a helpful diagnostic clue. Although patients with lambda light chains presented with more Bence Jones proteinuria, this did not correlate with the severity of initial renal functional impairment or with survival when compared to patients with kappa light chains. No other clinical or laboratory observation differentiated the groups with kappa light chains from those with lambda light chains. Amyloid was identified in seven patients. Their course was dominated by the features of primary systemic amyloidosis instead of the usual findings of classic myeloma. Patients with amyloidosis had lower initial serum albumin levels, fewer lytic bone lesions and reduced survival compared to patients without amyloidosis.
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U2 - 10.1016/0002-9343(75)90496-9
DO - 10.1016/0002-9343(75)90496-9
M3 - Article
C2 - 1130419
AN - SCOPUS:0016691192
SN - 0002-9343
VL - 58
SP - 601
EP - 619
JO - The American Journal of Medicine
JF - The American Journal of Medicine
IS - 5
ER -