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Robert A Kyle - One of the best experts on this subject based on the ideXlab platform.

  • Bence Jones cryoglobulinuria characterization of a urinary κ light chain cryoglobulin
    Clinical Chemistry, 2006
    Co-Authors: Abdul Jaleel, Barbara A L Owen, Michelle K Manske, Jerry A Katzmann, Robert A Kyle, Roshini S Abraham
    Abstract:

    Bence Jones Protein with cryoglobulin properties is rare. One of the earliest reports of Bence Jones cryoglobulinuria was made by Alper in 1966 (1) on a patient with multiple myeloma who had a λ Bence Jones cryoglobulin (cryo) in the urine. In this letter, we report a patient with κ Bence Jones cryoglobulinuria. We performed physicochemical analysis of the cryo Protein and compared it with a noncryo monoclonal urinary κ light chain to improve our understanding of the molecular basis of urine cryoglobulin formation. The patient was a 73-year-old male who had clinical and laboratory findings consistent with multiple myeloma. Serum immunofixation revealed a monoclonal κ light chain. Urine Protein electrophoresis and immunofixation of a randomly collected urine sample revealed the presence of a monoclonal κ Bence Jones Protein. This urine sample formed a gel at 4 °C, which disappeared when the sample was warmed to 37 °C. As a control, we used the urine from a myeloma patient with κ Bence Jones Protein without cryo formation. We performed single-dimension sodium dodecyl sulfate–polyacrylamide gel electrophoresis (SDS-PAGE) on the control and cryo urine samples, which revealed a single band …

  • monoclonal gammopathy of undetermined significance
    Clinical Lymphoma Myeloma & Leukemia, 2005
    Co-Authors: Robert A Kyle, Vincent S Rajkumar
    Abstract:

    Monoclonal gammopathy of undetermined significance (MGUS) is characterized by the presence of a serum monoclonal Protein (M-Protein) less than 3 g/dL; fewer than 10 percent plasma cells in the bone marrow; no or only small amounts of M-Protein (Bence Jones Protein) in the urine; absence of lytic lesions, anemia, hypercalcemia, and renal insufficiency; and most importantly, stability of the M-Protein and failure of development of other abnormalities. Detection of an M-Protein is accomplished by electrophoresis on agarose or cellulose acetate followed by immunoelectrophoresis or immunofixation. The latter is more sensitive and is particularly useful when one is searching for a small M-Protein (Kyle, 1994).

  • correlation of serum immunoglobulin free light chain quantification with urinary Bence Jones Protein in light chain myeloma
    Clinical Chemistry, 2002
    Co-Authors: Roshini S Abraham, Robert A Kyle, Raynell J Clark, Sandra C Bryant, James F Lymp, Timothy S Larson, Jerry A Katzmann
    Abstract:

    Multiple myeloma is a malignant plasma cell dyscrasia characterized by bone marrow plasmacytosis (1). Malignant plasma cells produce an abnormal monoclonal immunoglobulin, the laboratory hallmark of the disease process, as well as cytokines, which stimulate cells of the bone marrow microenvironment (2)(3). The neoplastic clone and its products cause the dysfunction of several organs, including bone pain or fractures, renal failure, anemia, susceptibility to infection, hyperviscosity, and hypercalcemia (1). However, the most characteristic feature of multiple myeloma and other monoclonal gammopathies is the presence of a serum and/or urinary monoclonal (M) component on immunofixation (4). Approximately two-thirds of patients with a serum M component also have Bence Jones Proteins (BJPs) in the urine. In almost 20% of myelomas, only immunoglobulin light chains are present in the serum and/or urine and are often designated as light chain multiple myeloma (LCMM). Renal failure occurs in ∼25% of myeloma patients, and there is some renal pathology in more than one-half (1)(5)(6). The number of patients with renal disease varies considerably depending on the criteria used to define renal impairment. Serum creatinine concentrations remain in the reference interval until the glomerular filtration rate is reduced by almost 50%; therefore, the data obtained with blood creatinine concentrations most likely underestimate the incidence of renal involvement in myeloma. Data using the estimated creatinine clearance rate (7), which takes into account various variables, including patient age, weight, and gender, indicate that approximately one-half of patients have renal insufficiency at the time of diagnosis (5). The nature of the M component is also associated with the prevalence of renal disease (5)(8)(9). In light chain myeloma, 65% of patients have impaired renal function at the time of diagnosis when estimated creatinine clearance is used as the variable (5). The …

  • Immunoglobulin D Multiple Myeloma: Presenting Features, Response to Therapy, and Survival in a Series of 53 Cases
    1994
    Co-Authors: Joan Blad, John A. Lust, Robert A Kyle
    Abstract:

    acteristics, response to therapy, and survival in 53 pa-tients with immunoglobulin D (IgD) multiple myeloma (MM) from a single institution. Patients and Methods: Records of all Mayo Clinic pa-tients with IgD MM seen between January 1, 1965 and December 31, 1992 were reviewed. Survival curves were plotted according to the Kaplan-Meier method and statistically compared using the log-rank test. Results: The main presenting features were bone pain (72%), ftigue (36%), weight loss (32%), extramedullary plasmacytomas (19%), and associated amyloidosis (19%). Renal function impairment and hypercalcemia were present in 33 % and 22 % of patients, respectively. The serum electrophoretic pattern showed an M-spike in only 60 % of the patients, the remaining having either hypogammaglobulinemia or a normal-appearing pat-tern. Bence Jones Proteinuria was identified in 96%. The type of light chain was A in 60 % of the patients, M ULTIPLE MYELOMA (MM) is characterized by the neoplastic proliferation of a single clone of plasma cells that produce a monoclonal immunoglobulin (Ig). According to the type of monoclonal Protein (M-Protein) produced, the most common immunologic type of MM is IgG, followed by IgA and light-chain only (Bence Jones Protein). In comparison, IgD myeloma, first recognized by Rowe and Fahey, ' affects less than 2 % o

Jerry A Katzmann - One of the best experts on this subject based on the ideXlab platform.

  • Bence Jones cryoglobulinuria characterization of a urinary κ light chain cryoglobulin
    Clinical Chemistry, 2006
    Co-Authors: Abdul Jaleel, Barbara A L Owen, Michelle K Manske, Jerry A Katzmann, Robert A Kyle, Roshini S Abraham
    Abstract:

    Bence Jones Protein with cryoglobulin properties is rare. One of the earliest reports of Bence Jones cryoglobulinuria was made by Alper in 1966 (1) on a patient with multiple myeloma who had a λ Bence Jones cryoglobulin (cryo) in the urine. In this letter, we report a patient with κ Bence Jones cryoglobulinuria. We performed physicochemical analysis of the cryo Protein and compared it with a noncryo monoclonal urinary κ light chain to improve our understanding of the molecular basis of urine cryoglobulin formation. The patient was a 73-year-old male who had clinical and laboratory findings consistent with multiple myeloma. Serum immunofixation revealed a monoclonal κ light chain. Urine Protein electrophoresis and immunofixation of a randomly collected urine sample revealed the presence of a monoclonal κ Bence Jones Protein. This urine sample formed a gel at 4 °C, which disappeared when the sample was warmed to 37 °C. As a control, we used the urine from a myeloma patient with κ Bence Jones Protein without cryo formation. We performed single-dimension sodium dodecyl sulfate–polyacrylamide gel electrophoresis (SDS-PAGE) on the control and cryo urine samples, which revealed a single band …

  • correlation of serum immunoglobulin free light chain quantification with urinary Bence Jones Protein in light chain myeloma
    Clinical Chemistry, 2002
    Co-Authors: Roshini S Abraham, Robert A Kyle, Raynell J Clark, Sandra C Bryant, James F Lymp, Timothy S Larson, Jerry A Katzmann
    Abstract:

    Multiple myeloma is a malignant plasma cell dyscrasia characterized by bone marrow plasmacytosis (1). Malignant plasma cells produce an abnormal monoclonal immunoglobulin, the laboratory hallmark of the disease process, as well as cytokines, which stimulate cells of the bone marrow microenvironment (2)(3). The neoplastic clone and its products cause the dysfunction of several organs, including bone pain or fractures, renal failure, anemia, susceptibility to infection, hyperviscosity, and hypercalcemia (1). However, the most characteristic feature of multiple myeloma and other monoclonal gammopathies is the presence of a serum and/or urinary monoclonal (M) component on immunofixation (4). Approximately two-thirds of patients with a serum M component also have Bence Jones Proteins (BJPs) in the urine. In almost 20% of myelomas, only immunoglobulin light chains are present in the serum and/or urine and are often designated as light chain multiple myeloma (LCMM). Renal failure occurs in ∼25% of myeloma patients, and there is some renal pathology in more than one-half (1)(5)(6). The number of patients with renal disease varies considerably depending on the criteria used to define renal impairment. Serum creatinine concentrations remain in the reference interval until the glomerular filtration rate is reduced by almost 50%; therefore, the data obtained with blood creatinine concentrations most likely underestimate the incidence of renal involvement in myeloma. Data using the estimated creatinine clearance rate (7), which takes into account various variables, including patient age, weight, and gender, indicate that approximately one-half of patients have renal insufficiency at the time of diagnosis (5). The nature of the M component is also associated with the prevalence of renal disease (5)(8)(9). In light chain myeloma, 65% of patients have impaired renal function at the time of diagnosis when estimated creatinine clearance is used as the variable (5). The …

Roshini S Abraham - One of the best experts on this subject based on the ideXlab platform.

  • Bence Jones cryoglobulinuria characterization of a urinary κ light chain cryoglobulin
    Clinical Chemistry, 2006
    Co-Authors: Abdul Jaleel, Barbara A L Owen, Michelle K Manske, Jerry A Katzmann, Robert A Kyle, Roshini S Abraham
    Abstract:

    Bence Jones Protein with cryoglobulin properties is rare. One of the earliest reports of Bence Jones cryoglobulinuria was made by Alper in 1966 (1) on a patient with multiple myeloma who had a λ Bence Jones cryoglobulin (cryo) in the urine. In this letter, we report a patient with κ Bence Jones cryoglobulinuria. We performed physicochemical analysis of the cryo Protein and compared it with a noncryo monoclonal urinary κ light chain to improve our understanding of the molecular basis of urine cryoglobulin formation. The patient was a 73-year-old male who had clinical and laboratory findings consistent with multiple myeloma. Serum immunofixation revealed a monoclonal κ light chain. Urine Protein electrophoresis and immunofixation of a randomly collected urine sample revealed the presence of a monoclonal κ Bence Jones Protein. This urine sample formed a gel at 4 °C, which disappeared when the sample was warmed to 37 °C. As a control, we used the urine from a myeloma patient with κ Bence Jones Protein without cryo formation. We performed single-dimension sodium dodecyl sulfate–polyacrylamide gel electrophoresis (SDS-PAGE) on the control and cryo urine samples, which revealed a single band …

  • correlation of serum immunoglobulin free light chain quantification with urinary Bence Jones Protein in light chain myeloma
    Clinical Chemistry, 2002
    Co-Authors: Roshini S Abraham, Robert A Kyle, Raynell J Clark, Sandra C Bryant, James F Lymp, Timothy S Larson, Jerry A Katzmann
    Abstract:

    Multiple myeloma is a malignant plasma cell dyscrasia characterized by bone marrow plasmacytosis (1). Malignant plasma cells produce an abnormal monoclonal immunoglobulin, the laboratory hallmark of the disease process, as well as cytokines, which stimulate cells of the bone marrow microenvironment (2)(3). The neoplastic clone and its products cause the dysfunction of several organs, including bone pain or fractures, renal failure, anemia, susceptibility to infection, hyperviscosity, and hypercalcemia (1). However, the most characteristic feature of multiple myeloma and other monoclonal gammopathies is the presence of a serum and/or urinary monoclonal (M) component on immunofixation (4). Approximately two-thirds of patients with a serum M component also have Bence Jones Proteins (BJPs) in the urine. In almost 20% of myelomas, only immunoglobulin light chains are present in the serum and/or urine and are often designated as light chain multiple myeloma (LCMM). Renal failure occurs in ∼25% of myeloma patients, and there is some renal pathology in more than one-half (1)(5)(6). The number of patients with renal disease varies considerably depending on the criteria used to define renal impairment. Serum creatinine concentrations remain in the reference interval until the glomerular filtration rate is reduced by almost 50%; therefore, the data obtained with blood creatinine concentrations most likely underestimate the incidence of renal involvement in myeloma. Data using the estimated creatinine clearance rate (7), which takes into account various variables, including patient age, weight, and gender, indicate that approximately one-half of patients have renal insufficiency at the time of diagnosis (5). The nature of the M component is also associated with the prevalence of renal disease (5)(8)(9). In light chain myeloma, 65% of patients have impaired renal function at the time of diagnosis when estimated creatinine clearance is used as the variable (5). The …

Christian Cambillau - One of the best experts on this subject based on the ideXlab platform.

  • The structure of an entire noncovalent immunoglobulin kappa light-chain dimer (Bence-Jones Protein) reveals a weak and unusual constant domains association.
    European journal of biochemistry, 1999
    Co-Authors: Alain Roussel, Silvia Spinelli, Sophie Deret, Jorge Navaza, Pierre Aucouturier, Christian Cambillau
    Abstract:

    Monoclonal free light chains secreted in immunoproliferative disorders are frequently involved in renal complications, including a specific proximal tubule impairment, Fanconi's syndrome. The latter is characterized in most cases by intracellular crystallization including a light-chain variable-domain fragment which resists lysosomal proteases. Bence-Jones Protein (BJP) DEL was isolated from a patient with myeloma-associated Fanconi's syndrome. The crystal structure of this human kappa immunoglobulin light-chain noncovalent dimer was determined using molecular replacement with the structure of molecule REI, as the variable domain, and that of BJP LOC as the constant domain. To our knowledge, DEL is the first complete kappa BJP structure described to date. The R-factor is 20.7% at 2.8 A resolution. The BJP DEL dimer was compared with other light-chain dimers and with Fab fragments with a kappa light chain. Although the domain-folding pattern was similar, the relative positions of the constant domains differed. BJP DEL showed a noncanonical quaternary structural arrangement which may be attributable to the poor CL-CL affinity and lack of an interchain disulfide bridge, combined with the conformational editing effect of the crystal-packing forces. Our results suggest that, in the absence of a disulfide bridge, most BJP CLs are probably mobile in solution. This may explain their high susceptibility to proteases and the absence of naturally occurring crystals for these dimers. Furthermore, these findings of an unusual quaternary structure of an immunoglobulin light-chain association extend our knowledge about the large and highly diverse structures of the immunoglobulin superfamily.

Oliveira B. - One of the best experts on this subject based on the ideXlab platform.

  • Tryptic Peptides Of A Human Bence Jones Protein Jjo: Partial Characterization Of The C Domain
    2015
    Co-Authors: Marques M.r.f., Marangoni S., Oliveira B.
    Abstract:

    Information is provided regarding the partial characterization of the lambda constant region of Bence Jones Protein JJO. Through the isolation and characterization of defined C λ domain tryptic peptides bearing key residues which may be associated with characteristic serological phenotypes, we suggest one of the following arrangements for the JJO Protein: Oz(-) Kern(-) Mcg(-) Weir(-) Ev(-) Way(-) Mz(-) or Oz(-) Kern(+) Mcg(-) Weir(-) Ev(-) Way(-) Mz(-). The dispositions proposed could tentatively indicate two (Oz- Kern- and Oz- Kern+) of the described four non-allelic forms of the human lambda constant region light chain.15312312

  • Chemical Studies Of A Urinary Bence Jones Protein; Amino Acid Composition And Evidence Of A Covalently Bound Dimeric Structure
    2015
    Co-Authors: Marques M.r.f., Marangoni S., Oliveira B.
    Abstract:

    Bence Jones Protein (JJO) typed as λ by specific immune sera was isolated from the urine of a paraProteinemic patient. Amino acid composition of BJP (JJO) showed a total number of 206 residues excluding tryptophan; the value found for half cystine determined as cysteic acid was 5 moles per mole of Protein (taken as 22600). This result suggests that BJP (JJO) is arranged as a dimer in urine. Under 10% PAGE/SDS, native and reduced BJP (JJO) migrate as single bands. Native Protein displays an electrophoretic mobility close to H chains (50 K) of reduced IgG1(JJO) λ and IgG1(GOB) k paraProteins; reduced BJP migrates as a typical lambda chain monomer (25 K) obtained from reduced IgG1(JJO) and IgG3(FKW) paraProteins. The data presented in compatible with the interpretation that BJP (JJO) in urine consists of two covalently bound homologous λ chains in a dimeric configuration.12111008100

  • The Variable Region Of A Human Bence Jones Protein (jjo): Assignment To The V λ Iii Subgroup
    2015
    Co-Authors: Marques M.r.f., Marangoni S., Oliveira B.
    Abstract:

    Results from methanolysis of Bence Jones JJO Protein followed by dinitrophenylation allowed us to identify seven DNP derivatives, one of them showing a clearly more intensive colour corresponding to di-DNP-tyrosine. These data indicated that Protein JJO should not present PCA as an N-terminal residue. The knowledge that N-terminal tyrosine is a characteristic residue of V λ III domains allowed us to tentatively assign the BJP (JJO) variable region as a member of the V λ III sub type. Through Ouchterlony analysis Bence Jones (JJO) Protein gives a spur over another λ Protein (BJP Mau) using as reagent rabbit antiserum to BJP (JJO). These data could lead to a preliminary but inconclusive speculation that BJP (JJO) is associated with antigenic sub type St-III+ as described for other V λ III sub types.15312112