Macroglobulinemia with Bence Jones Proteinuria: Comparison of Urinary Protein and L Chain of Serum Proteins*

Abstract

Edelman and Gally (1) have recently shown that an individual's Bence Jones proteins are chemically and physically similar to the low molecular weight polypeptides (L chains) obtained by reductive cleavage of his myeloma proteins. Indeed, Putnam (2) has shown that peptide maps of the tryptic digests of oxidized Bence Jones proteins correspond to a portion of the peptide map of similarly treated autologous serum myeloma protein.Edelman and Benacerraf (3) have postulated that the manufacture of the L polypeptide chains is asynchronous in multiple myeloma, and thus a part of the cell output appears in the urine as Bence Jones protein, whereas other chains are incorporated into the circulating myeloma globulin. It was of interest, therefore, to examine the uncommon instance of Bence Jones proteinuria in a patient with Waldenstrdm's macroglobulinemia and particularly to compare the L chains of the serum macroglobulins and y2-globulins with the urinary Bence Jones protein.Evidence is presented to show that the Bence Jones protein is chemically and antigenically similar to the L chain derived from the patient's macroglobulin and antigenically deficient when compared to the L chain of the autologous 7 S y2-globulin.Materials and Methods Clinical informationt. The patient in the present study, a 74-year-old Caucasian male, was admitted to the Ear, Nose, and Throat Service of the University of California * Submitted for publication July 16, 1963; accepted September 19, 1963. Supported in part by grants A-1229 and B-1099 from the National Institutes of Health, Bethesda, Md., a grant from The National Foundation, and funds from the Research Committee of the University of California School of Medicine.Medical Center, in January 1963, because of gradual, progressive loss of hearing during the preceding year. He had always been in excellent health except for several episodes of unexplained epistaxis one year before admission. In the course of the initial physical examination, hepatomegaly was detected, and initial laboratory studies revealed a 3 + proteinuria positive for Bence Jones protein and a normochromic, normocytic anemia with marked rouleaux formation. Additional laboratory studies showed a hemoglobin of 9.3 g per 100 ml, hematocrit of 29%, and leukocyte count of 5,100 per mm'. The total serum protein was 11.4 g per 100 ml; serum electrophoretic analysis showed 20% albumin and 61% y-globulin. A Sia test showed a 4+ reaction. Bone marrow aspiration detected lymphocytosis and plasmacytosis, and an X-ray bone survey demonstrated generalized demineralization.Materials. The sodium salts of reagent grade phosphate and acetate were used for the preparation of the buffers; reagent grade urea was used. Additional materials were mercaptoethanol,1 iodoacetamide,2 carboxymethyl-cellulose (CM-cellulose, 0.8 mEq per g),3 and diethylaminoethyl-cellulose (DEAE-cellulose, 0.9 mEq per g).3 The exchangers were recycled through acid and base washes before use. Also used were cross-linked dextran (140 to 400 mesh) 4 and a crystalline prep...

Pernicious Anaemia, Paraproteinaemia With Unusual Features, and Chromosome Aberrations

“…In primary macroglobulinaemia the Bence-Jones protein excreted in the urine belongs to the Lchain type which also is found in most patients with multiple myeloma (10,16). In the present case the gamma-globulin component present in the urine seems similarly to be of the Kappa-chain type, which also was found in the serum para-…”