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Acquired C 1-inhibitor deficiency with angioedema due to pleomorphic immunocytoma in a patient with three malignant tumors: long-term follow-up data and presentation of an additional case

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Abstract

 Two cases of lymphoma-associated acquired C 1-inhibitor deficiency are described. In both patients, C 1-inhibitor deficiency and related symptoms preceded the diagnosis of the underlying neoplasm by several months. C 1-inhibitor deficiency was most likely due to consumption following immunocomplex formation. In both patients, a close relationship between low levels of C 1-inhibitor and tumor relapse was observed during follow-up. These findings indicate that measurement of C 1-inhibitor and complement factor C4 can be used as markers of disease activity in affected patients.

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Authors and Affiliations

  1. Department of Internal Medicine, Katharinenhospital, Kriegsbergstrasse 60, D-70714 Stuttgart, Germany, , , , , , DE

    W. Bohle, U. Rüther & P. Jipp

  2. Department of Internal Medicine, Eberhard-Karls-University, Otfried-Müller-Strasse 60, D-72076 Tübingen, Germany, , , , , , DE

    C. Bokemeyer

  3. Medical Practice, Kammerweg 1, D-72762 Reutlingen, Germany, , , , , , DE

    C. Nunnensiek

  4. Department of Internal Medicine, Kreiskrankenhaus, Erichsenweg 16, D-25813 Husum, Germany, , , , , , DE

    H. Haake & K. Feldmann

  5. Institute for Laboratory Medicine, Eichertstrasse 3, D-73035 Göppingen, Germany, , , , , , DE

    H. A. G. Müller

Authors
  1. W. Bohle
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  2. U. Rüther
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  3. C. Bokemeyer
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  4. C. Nunnensiek
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  5. H. A. G. Müller
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  6. H. Haake
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  7. K. Feldmann
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  8. P. Jipp
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Received: 21 December 1995 / Accepted: 12 February 1996

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Bohle, W., Rüther, U., Bokemeyer, C. et al. Acquired C 1-inhibitor deficiency with angioedema due to pleomorphic immunocytoma in a patient with three malignant tumors: long-term follow-up data and presentation of an additional case. Ann Hematol 72, 383–386 (1996). https://doi.org/10.1007/s002770050191

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  • DOI: https://doi.org/10.1007/s002770050191

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