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Diagnosis of Richter transformation in chronic lymphocytic leukemia: histology tips the scales

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Abstract

Development of diffuse large B-cell lymphoma in chronic lymphocytic leukemia, so-called Richter transformation (RT), occurs in 2–5% of patients and is associated with poor outcome. The clinical features of RT are fairly non-specific and unable to discriminate transformation from other mimics. In case of clinically suspected RT, a CT/MRT is recommended, and FDG-PET/CT may help to select the site of biopsy. Radiological features suggestive of RT have been defined, but there are only limited data about their predictive value, and histological confirmation is still considered the gold standard for RT diagnosis. We retrospectively analyzed 34 patients with clinically suspected RT and available radiological and histological data. A histopathological diagnosis of RT with concordant clinical and radiological findings was obtained in 13 patients. In 18 patients, CT did not show features of transformation, concordant with lack of RT in the biopsy. Of interest, a distinct lymphoma other than DLBCL was identified in two of these cases. A false-positive radiological diagnosis of RT was rendered in two patients, including a case of Herpes simplex virus lymphadenitis. In conclusion, our findings confirm the central role of tissue biopsy in the diagnostic work up in case of clinically suspected RT.

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Funding

BF is supported by the TÜFF-program, University of Tübingen (project 2320-0-0).

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Author notes

  1. Marius S. Horger and Falko Fend are both senior authors of this manuscript.

Authors and Affiliations

  1. Institute of Pathology and Neuropathology, University Hospital and Comprehensive Cancer Center Tübingen, University of Tübingen, Liebermeisterstr. 8, 72076, Tübingen, Germany

    Birgit Federmann, Julia Steinhilber & Falko Fend

  2. Department of Hematology and Oncology, University Hospital and Comprehensive Cancer Center Tübingen, University of Tübingen, 72076, Tübingen, Germany

    Martin R. Mueller

  3. Department of Radiology, University Hospital and Comprehensive Cancer Center Tübingen, University of Tübingen, 72076, Tübingen, Germany

    Marius S. Horger

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  1. Birgit Federmann
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  2. Martin R. Mueller
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  3. Julia Steinhilber
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Correspondence to Falko Fend.

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Federmann, B., Mueller, M.R., Steinhilber, J. et al. Diagnosis of Richter transformation in chronic lymphocytic leukemia: histology tips the scales. Ann Hematol 97, 1859–1868 (2018). https://doi.org/10.1007/s00277-018-3390-x

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  • DOI: https://doi.org/10.1007/s00277-018-3390-x

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