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J-chain-producing immunoblastic lymphoma in a case of Richter's syndrome

Immunohistochemical evidence for a gradual malignant transformation of a single B-cell clone and flow cytophotometric data

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Summary

A 66-year old male with Richter's syndrome died 52 month after diagnosis of chronic lymphocytic leukaemia (CLL). The clinical course was characterized by a marked IgM hypoglobulinaemia which paralleled a chronically relapsing Herpes simplex infection. Autopsy showed a large retroperitoneal and intraabdominal tumour mass and well defined supradiaphragmatic lymphomas. Histological examination revealed a composite tumour consisting of CLL B-cell type (B-CLL) and immunoblastic malignant lymphoma of B-cell type (B-IbL). The lymphocytes bear μ-chains on their surface and to a lesser extend within their cytoplasm, the obviously defective immunoblasts produce J chains exclusively. Flow cytophotometric data seem to indicate an identical diploid stem line of the two tumours. The majority of the cells are in G0/1 phase. The CLL rarely produces mitoses, however, the IbL has a mitotic rate of 7% and a considerable proportion (33%) of cells in the phase of DNA-synthesis. This is the fourth malignant lymphoma and the second immunoblastic lymphoma to be reported that produces J chain in the absence of immunoglobulin.

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Authors and Affiliations

  1. Institut für Allgemeine Pathologie und Pathologische Anatomie, Im Neuenheimer Feld 220, D-6900, Heidelberg

    P. Möller & B. Schüle

  2. Institut für Experimentelle und Vergleichende Pathologie, Federal Republic of Germany

    G. E. Feichter & D. Haag

  3. Medizinische Klinik der Universität Heidelberg, Federal Republic of Germany

    D. Fritze

Authors
  1. P. Möller
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  2. G. E. Feichter
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  3. D. Fritze
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  4. D. Haag
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  5. B. Schüle
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Möller, P., Feichter, G.E., Fritze, D. et al. J-chain-producing immunoblastic lymphoma in a case of Richter's syndrome. Virchows Arch. A Path. Anat. and Histol. 396, 213–224 (1982). https://doi.org/10.1007/BF00431242

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