title: Evans syndrome secondary to chronic lymphocytic leukaemia: presentation, treatment, and outcome.
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.
authors.affiliation: Department of Cell Therapy and Haematology, San Bortolo Hospital, Via Rodolfi 37, 36100, Vicenza, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Carli
authors.affiliation: Department of Cell Therapy and Haematology, San Bortolo Hospital, Via Rodolfi 37, 36100, Vicenza, Italy. carlovisco@hotmail.com.
authors.forename: Carlo
authors.initials: C
authors.lastname: Visco
authors.affiliation: Department of Cell Therapy and Haematology, San Bortolo Hospital, Via Rodolfi 37, 36100, Vicenza, Italy.
authors.forename: Erika
authors.initials: E
authors.lastname: Falisi
authors.affiliation: Department of Cell Therapy and Haematology, San Bortolo Hospital, Via Rodolfi 37, 36100, Vicenza, Italy.
authors.forename: Omar
authors.initials: O
authors.lastname: Perbellini
authors.affiliation: Department of Cell Therapy and Haematology, San Bortolo Hospital, Via Rodolfi 37, 36100, Vicenza, Italy.
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Novella
authors.affiliation: Department of Cell Therapy and Haematology, San Bortolo Hospital, Via Rodolfi 37, 36100, Vicenza, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Giaretta
authors.affiliation: Department of Medicine, Section of Haematology, University of Verona, Verona, Italy.
authors.forename: Isacco
authors.initials: I
authors.lastname: Ferrarini
authors.affiliation: Department of Immunohaematology, Transfusion Medicine and Human Genetics, San Bortolo Hospital, Vicenza, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Sandini
authors.affiliation: Department of Immunohaematology, Transfusion Medicine and Human Genetics, San Bortolo Hospital, Vicenza, Italy.
authors.forename: Alberta
authors.initials: A
authors.lastname: Alghisi
authors.affiliation: Department of Medicine, Section of Haematology, University of Verona, Verona, Italy.
authors.forename: Achille
authors.initials: A
authors.lastname: Ambrosetti
authors.affiliation: Department of Cell Therapy and Haematology, San Bortolo Hospital, Via Rodolfi 37, 36100, Vicenza, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Rodeghiero
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis* / Q000209:etiology* / Q000628:therapy; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage; D011379:Prognosis; D012189:Retrospective Studies; D013921:Thrombocytopenia / Q000175:diagnosis* / Q000209:etiology* / Q000628:therapy; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone
keywords: Autoimmune haemolytic anaemia; CLL; Chronic lymphocytic leukaemia; Evans syndrome; Immune thrombocytopenia