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Evans syndrome secondary to chronic lymphocytic leukaemia: presentation, treatment, and outcome

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Abstract

Evans syndrome (ES) is defined by the combination (either simultaneous or sequential) of immune thrombocytopenia (ITP) and autoimmune haemolytic anaemia (AIHA). When related to secondary conditions, ES may arise in patients with chronic lymphocytic leukaemia (CLL), which is frequently associated to autoimmune cytopenias (AIC). We analysed 25 patients with ES secondary to CLL, which were identified from a large series of consecutive patients with CLL, diagnosed and followed up in two institutions. They represented 2.9 % of the whole series. Thirteen patients presented with concurrent ITP and AIHA (simultaneous ES), while others developed the two AIC sequentially. Occurrence of ES was associated with unfavourable biological prognostic factors like ZAP-70 expression, unmutated immunoglobulin heavy chain variable region gene status, 17-p13 deletion and TP53 gene mutations. Of note, the majority of patients with ES (66 %) had stereotyped B cell receptor configuration. Most patients had short-lasting remissions and required second-line treatments to control the autoimmune manifestations of ES. Patients with ES were associated with inferior survival compared to patients not developing AIC, especially when ES developed early in the course of CLL, although the reduced survival was not confirmed by multivariate analysis. In conclusion, ES secondary to CLL is a difficult-to-treat complication, characterised by adverse biological features and clinical outcome.

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Acknowledgments

This work was supported in part by: AViLL/AIL (Associazione Vicentina per le Leucemie, i Linfomi e il Mieloma/Associazione Italiana Leucemie) (Vicenza, Italy); Hematology Project Foundation (HPF, Fondazione Progetto Ematologia, Vicenza, Italy); Regione Veneto, Italy, through the “Ricerca Sanitaria Finalizzata 2006” program; Associazione Italiana Ricerca sul Cancro (AIRC). We thank Dr. Andrea Timillero for his precious editing assistance.

GC, CV, EF, OP, and IF analysed the data and performed the research; GC and CV wrote the paper; CV designed the research study; EN, IG, AS, and AAL contributed essential reagents or tools; AAM and FR revised the paper.

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Authors and Affiliations

  1. Department of Cell Therapy and Haematology, San Bortolo Hospital, Via Rodolfi 37, 36100, Vicenza, Italy

    Giuseppe Carli, Carlo Visco, Erika Falisi, Omar Perbellini, Elisabetta Novella, Ilaria Giaretta & Francesco Rodeghiero

  2. Department of Medicine, Section of Haematology, University of Verona, Verona, Italy

    Isacco Ferrarini & Achille Ambrosetti

  3. Department of Immunohaematology, Transfusion Medicine and Human Genetics, San Bortolo Hospital, Vicenza, Italy

    Alessandra Sandini & Alberta Alghisi

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  1. Giuseppe Carli
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  2. Carlo Visco
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  3. Erika Falisi
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  4. Omar Perbellini
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  5. Elisabetta Novella
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  11. Francesco Rodeghiero
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Correspondence to Carlo Visco.

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Carli, G., Visco, C., Falisi, E. et al. Evans syndrome secondary to chronic lymphocytic leukaemia: presentation, treatment, and outcome. Ann Hematol 95, 863–870 (2016). https://doi.org/10.1007/s00277-016-2642-x

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  • DOI: https://doi.org/10.1007/s00277-016-2642-x

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