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H-Kettenkrankheit: Humorale und celluläre Befunde bei sechs Fällen vom μ-Kettentyp

Heavy chain disease: Humoral and cellular findings in six patients with μ chain disease

  • Original Papers
  • Clinical Oncology or Epidemiology
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Summary

Clinical and chemical findings in 6 patients with μ-chain disease are reported. In all of these patients a lymphoproliferative disease was observed which by its symptoms resembled CLL. This similarity was underlined by the finding of an impaired response of PBL of the patients to mitogens as PHA, PWM, and Con A. A substantial result of our studies lies in the fact that the presence of μ-chains in the serum does not correlate with a clinically defined entity. Moreover, μ-chains have been observed in a case of benign lymphadenopathy. In one case a good remission of 4 years duration could be induced by the application of cytostatic agents.

Zusammenfassung

Es werden klinische und eiweißchemische Befunde bei 6 Patienten mit μ-Kettenkrankheit mitgeteilt. Bei allen Patienten bestand ein lymphoproliferatives Syndrom, das klinisch am ehesten der Chronischen Lymphatischen Leukämie bzw. einem Non-Hodgkin-Lymphom niedrigen Malignitätsgrades entsprach. Die Ähnlichkeit mit der CLL wird durch den Befund einer eingeschränkten Stimulierbarkeit von PBL dieser Fälle mit mitogenen Substanzen wie PHA, PWM und Con A unterstrichen. Freie μ-Ketten im Serum sind keiner einheitlichen, klinisch von anderen lymphoproliferativen Erkrankungen abzugrenzenden Symptomatik zuzuordnen und können auch im Rahmen einer gutartigen Lymphadenopathie auftreten. In einem Fall konnte durch eine kombinierte Chemotherapie eine über 4 Jahre andauernde Remission erzielt werden.

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Literatur

  • Abdou, N.L., Abdou, N.I.: Immunoglobulin receptors on human leukocytes. IV. Differences between bone marrow and blood cells in multiple myeloma and chronic lymphocytic leukemia: Effects of therapy. J. Lab. Clin. Med. 82, 611 (1973)

    Google Scholar 

  • Abdou, N.I., Abdou, N.L.: The monoclonal nature of lymphocytes in multiple myeloma. Ann. Intern. Med. 83, 42 (1975)

    Google Scholar 

  • Adams-Mayne, M.E., Jirgensons, B.: Purification and characterization of four Bence-Jones proteins. Arch. Biochem. Biophys. 113, 575 (1966)

    Google Scholar 

  • Baglioni, C., Cioli, D., Gorini, G., Ruffilli, A., Alescio-Zonta, L.: Studies on fragments of light chains of human immunoglobulins: Genetic and biochemical implications. Cold Spring Harbor Symp. Quant. Biol. 32, 147 (1967)

    Google Scholar 

  • Bernier, G.M., Putnam, F.W.: Monomer-dimer forms of Bence-Jones proteins. Nature 200, 223 (1963)

    Google Scholar 

  • Bøyum, A.: Separation of leucocytes from blood and bone marrow. Scand. J. Clin. Lab. Invest. 21, Suppl. 97 (1968)

    Google Scholar 

  • DeVita, V.T. Jr., Serpick, A.A., Carbone, P.P.: Combination chemotherapy in the treatment of advanced Hodgkin's disease. Ann. Intern. Med. 73, 881 (1970)

    Google Scholar 

  • Douglas, S.D., Kamin, R.M., Fudenberg, H.H.: Human lymphocyte response to phytomitogens in vitro: Normal, agammaglobulinemic and paraproteinemic individuals. J. Immunol. 103, 1185 (1969)

    Google Scholar 

  • Du Bois, M.J.G.J., Huismans, D.R., Schellkens, P.Th.A., Eijsvoogel, V.P.: Investigation and standardization of the conditions for micro-lymphocyte cultures. Tissue Antigens 3, 402 (1973)

    Google Scholar 

  • Edelman, G.M., Gally, J.A.: The nature of Bence Jones proteins. J. Exp. Med. 116, 207 (1962)

    Google Scholar 

  • Forte, F.A., Prelli, F., Yount, W.J., Jerry, L.M., Kochwa, Sh., Franklin, E.C. Kunkel, H.G.: Heavy chain disease of the μ (γM) type: Report of the first case. Blood 36, 137 (1970)

    Google Scholar 

  • Fragione, B., Franklin, E.C.: Heavy chain disease: Clinical features and molecular significance of the disordered immunoglobulin structure. Semin. Hematol. 10, 53 (1973)

    Google Scholar 

  • Franklin, E.C., Lowenstein, J., Bigelow, B., Meltzer, M.: Heavy chain disease — a new disorder of serum γ-globulins. Am. J. Med. 37, 332 (1964)

    Google Scholar 

  • Franklin, E.C.: μ-chain disease. Arch. Intern. Med. 135, 71 (1975)

    Google Scholar 

  • Janossy, G., Greaves, M.F., Doenhoff, M.J., Šnajdr, J.: Lymphocyte activation. V. Quantitation of the proliferative responses to mitogens using defined T and B cell populations. Clin. Exp. Immunol. 14, 581 (1973)

    Google Scholar 

  • Jondal, M., Holm, G., Wigzell, H.: Surface markers on human T and B lymphocytes. I. A large population of lymphocytes forming nonimmune rosettes with sheep red blood cells. J. Exp. Med. 136, 207 (1972)

    Google Scholar 

  • Jønsson, V.: Influence of prednisone and cytostatics on human blood B-, T- and O-lymphocytes in diseases. Scand. J. Haematol. 15, 109 (1975)

    Google Scholar 

  • Kaplan, M.E., Clark, C.: An improved rosetting assay for detection of human T-lymphocytes. J. Immunol. Methods 5, 131 (1974)

    Google Scholar 

  • Manousos, O.N., Economidou, J.C., Georgiadou, D.E., Pratsika-Ougourloglou, K.G., Hadziyannis, S.J., Merikas, G.E., Henry, K., Doe, W.F.: Alpha-chain disease with clinical, immunological, and histological recovery. Br. Med. J. 2, 409 (1974)

    Google Scholar 

  • McConahey, P.J., Dixon, F.J.: A method of trace iodination of proteins for immunologic studies. Int. Arch. Allergy Appl. Immunol. 29, 185 (1966)

    Google Scholar 

  • Mellstedt, H., Hammarström, S., Holm, G.: Monoclonal B-lymphocytes in human myelomatosis. Abstr. 11th Intern. Cancer Congr. Florence, Oct. 1974 1, 334 (1974)

    Google Scholar 

  • Reisfeld, R.A., Small, P.A., Jr.: Electrophoretic heterogeneity of polypeptide chains of specific antibodies. Science 152, 1253 (1966)

    Google Scholar 

  • Rogé, J., Druet, P., Marche, C., Silvereano de Roissard, F., Camilleri, J.P.: Alpha-chain disease cured with antibiotics. Br. Med. J. 4, 225 (1975)

    Google Scholar 

  • Rubin, A.D., Havemann, K., Dameshek, W.: Studies in chronic lymphocytic leukemia: Further studies of the proliferative abnormality of the blood lymphocyte. Blood 33, 313 (1969)

    Google Scholar 

  • Salmon, S.E., Seligmann, M.: B-cell neoplasia in man. Lancet II, 1230 (1974)

    Google Scholar 

  • Schellekens, P.T.A., Eijsvoogel, V.P.: Lymphocyte transformation in vitro. I. Tissue culture conditions and quantitative measurements. Clin. Exp. Immunol. 3, 571 (1968)

    Google Scholar 

  • Scheurlen, P.G.: Immunologische Untersuchungen an heterogen zusammengesetzten Plasmocytom-γ-Globulinen. Klin. Wochenschr. 41, 723 (1963)

    Google Scholar 

  • Scheurlen, P.G., Tischendorf, F.W.: Untersuchungen über die Dissoziation von γ1-Makroglobulinen durch Cysteamin. II. Elektrophoretische Befunde. Klin. Wochenschr. 43, 980 (1965)

    Google Scholar 

  • Schwarz, J.A., Jost, H., Pitrawirana, A., Scheurlen, P.G.: Monomeres IgM bei monoclonalen Gammopathien, Autoimmun- und Infektionserkrankungen. Z. Immunitaetsforsch. 147, 310 (1974)

    Google Scholar 

  • Schweitzer, M., Melief, C.J.M., Eijsvoogel, V.P.: The nature of the transforming lymphocyte in chronic lymphocytic leukemia. Eur. J. Immunol. 3, 121 (1973)

    Google Scholar 

  • Seligmann, M., Mihaesco, E., Hurez, D., Mihaesco, C., Preud'homme, J.-L., Rambaud, J.-C.: Immunochemical studies in four cases of alpha chain disease. J. Clin. Invest. 48, 2374 (1969)

    Google Scholar 

  • Shapiro, A.L., Viñuela, E., Maizel, J.V.: Molecular weight estimation of polypeptide chains by electrophoreses in SDS-polyacrylamide gels. Biochem. Biophys. Res. Comm. 28, 815 (1967)

    Google Scholar 

  • Smith, J.L., Cowling, D.C., Barker, C.R.: Response of lymphocytes in chronic lymphocytic leukaemia to plant mitogens. Lancet I, 229 (1972)

    Google Scholar 

  • Smithies, O.: An improved procedure for starch-gel electrophoresis. Further variations in the serum proteins of normal individuals. Biochem. J. 71, 585 (1959)

    Google Scholar 

  • Ternynck, T., Avrameas, S.: Polyacrylamide-protein immunoadsorbents prepared with glutaraldehyde. FEBS-Letters 23, 24 (1972)

    Google Scholar 

  • Weber, K., Osborn, M.: The reliability of molecular weight determinations by dodecyl sulfatepolyacrylamide gel electrophoresis. J. Biol. Chem. 244, 4406 (1969)

    Google Scholar 

  • Weiner, M.S., Bianco, C., Nussenzweig, V.: Enhanced binding of neuraminidase-treated sheep erythrocytes to human T lymphocytes. Blood 42, 939 (1973)

    Google Scholar 

  • Wetter, O., Van de Weert, M., Reis, H.E., Leiner, G.: Mitogenic stimulation of human leukemic plasma cells. Exp. Hematol. 2, 73 (1974)

    Google Scholar 

  • Wetter, O., Drosdziok, W., Müller, B.: Detection and preparation of immunoglobulin (Ig) μ chain protein in sera of patients with heavy chain diseases (HCD). Klin. Wochenschr. 53, 885 (1975)

    Google Scholar 

  • Wetter, O.: Alpha-chain disease cured with antibiotics. Br. Med. J. 1, 835 (1976)

    Google Scholar 

  • Wetter, O., Linder, K.H., Leene, W.: μ-Chain secretion by peripheral blood lymphocytes in μ-chain disease: functional and ultrastructural characterization of cells with μ-chain production. Eur. J. Cancer 13, 985 (1977)

    Google Scholar 

  • Wybran, J., Chantler, S., Fudenberg, H.H.: Isolation of normal T cells in chronic lymphatic leukaemia. Lancet I, 126 (1973)

    Google Scholar 

  • Zawadzki, Z.A., Benedek, T.G., Ein, D., Easton, J.M.: Rheumatoid arthritis terminating inheavychain disease. Ann. Intern. Med. 70, 335 (1969)

    Google Scholar 

  • Zucker-Franklin, D., Franklin, E.C.: Ultrastructural and immunofluorescence studies of the cells associated with μ-chain disease. Blood 37, 257 (1971)

    Google Scholar 

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Authors and Affiliations

  1. Westdeutsches Tumorzentrum, Innere Universitätsklinik (Tumorforschung), Hufelandstraße 55, D-4300, Essen, Bundesrepublik Deutschland

    O. Wetter, C. G. Schmidt, K. H. Linder & W. Leene

  2. Jan Swammerdam Institut, Universität Amsterdam, Amsterdam, Niederlande

    O. Wetter, C. G. Schmidt, K. H. Linder & W. Leene

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  1. O. Wetter
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  2. C. G. Schmidt
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  3. K. H. Linder
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  4. W. Leene
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Wetter, O., Schmidt, C.G., Linder, K.H. et al. H-Kettenkrankheit: Humorale und celluläre Befunde bei sechs Fällen vom μ-Kettentyp. J Cancer Res Clin Oncol 94, 207–223 (1979). https://doi.org/10.1007/BF00422499

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  • DOI: https://doi.org/10.1007/BF00422499

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