title: Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia: a GIMEMA study of 308 patients.
abstract: The clinical outcome, response to treatment, and occurrence of acute complications were retrospectively investigated in 308 primary autoimmune hemolytic anemia (AIHA) cases and correlated with serological characteristics and severity of anemia at onset. Patients had been followed up for a median of 33 months (range 12-372); 60% were warm AIHA, 27% cold hemagglutinin disease, 8% mixed, and 5% atypical (mostly direct antiglobulin test negative). The latter 2 categories more frequently showed a severe onset (hemoglobin [Hb] levels ≤6 g/dL) along with reticulocytopenia. The majority of warm AIHA patients received first-line steroid therapy only, whereas patients with mixed and atypical forms were more frequently treated with 2 or more therapy lines, including splenectomy, immunosuppressants, and rituximab. The cumulative incidence of relapse was increased in more severe cases (hazard ratio 3.08; 95% confidence interval, 1.44-6.57 for Hb ≤6 g/dL; P < .001). Thrombotic events were associated with Hb levels ≤6 g/dL at onset, intravascular hemolysis, and previous splenectomy. Predictors of a fatal outcome were severe infections, particularly in splenectomized cases, acute renal failure, Evans syndrome, and multitreatment (4 or more lines). The identification of severe and potentially fatal AIHA in a largely heterogeneous disease requires particular experienced attention by clinicians.
authors.affiliation: Unità Operativa Oncoematologia, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy;
authors.forename: Wilma
authors.initials: W
authors.lastname: Barcellini
authors.affiliation: Unità Operativa Oncoematologia, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy;
authors.forename: Bruno
authors.initials: B
authors.lastname: Fattizzo
authors.affiliation: Unità Operativa Oncoematologia, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy;
authors.forename: Anna
authors.initials: A
authors.lastname: Zaninoni
authors.affiliation: Unità Operativa Oncoematologia, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy;
authors.forename: Tommaso
authors.initials: T
authors.lastname: Radice
authors.affiliation: Dipartimento di Terapie Cellulari ed Ematologia, Ospedale San Bortolo, Vicenza, Italy;
authors.forename: Ilaria
authors.initials: I
authors.lastname: Nichele
authors.affiliation: Dipartimento di Terapie Cellulari ed Ematologia, Ospedale San Bortolo, Vicenza, Italy;
authors.forename: Eros
authors.initials: E
authors.lastname: Di Bona
authors.affiliation: Struttura Complessa a Direzione Universitaria Ematologia, Dipartimento di Medicina Traslazionale, Università del Piemonte Orientale Amedeo Avogadro, Novara, Italy;
authors.forename: Monia
authors.initials: M
authors.lastname: Lunghi
authors.affiliation: Unità Operativa Trasfusionale e Immunologia, Centro Regionale Malattie del Sangue, Castelfranco Veneto, Italy;
authors.forename: Cristina
authors.initials: C
authors.lastname: Tassinari
authors.affiliation: Ematologia, Università Degli Studi di Napoli Federico II, Napoli, Italy;
authors.forename: Fiorella
authors.initials: F
authors.lastname: Alfinito
authors.affiliation: Unità Operativa Ematologia, Ospedale Sant'Andrea, Facoltà di Medicina e Psicologia, Università "Sapienza" Roma, Roma, Italy;
authors.forename: Antonella
authors.initials: A
authors.lastname: Ferrari
authors.affiliation: Unità Operativa Ematologia, Ospedale Sant'Andrea, Facoltà di Medicina e Psicologia, Università "Sapienza" Roma, Roma, Italy;
authors.forename: Anna Paola
authors.initials: AP
authors.lastname: Leporace
authors.affiliation: Unità Operativa Ematologia, Ospedale S. Eugenio, Roma, Italy;
authors.forename: Pasquale
authors.initials: P
authors.lastname: Niscola
authors.affiliation: Unità Operativa Ematologia e Trapianto, Ospedale S. Gerardo di Monza e Università degli Studi di Milano Bicocca, Milano, Italy;
authors.forename: Monica
authors.initials: M
authors.lastname: Carpenedo
authors.affiliation: Unità Operativa Oncoematologia, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy;
authors.forename: Carla
authors.initials: C
authors.lastname: Boschetti
authors.affiliation: Unità Operativa Centro Trasfusionale e di Immunoematologia, and.
authors.forename: Nicoletta
authors.initials: N
authors.lastname: Revelli
authors.affiliation: Unità Operativa Centro Trasfusionale e di Immunoematologia, and.
authors.forename: Maria Antonietta
authors.initials: MA
authors.lastname: Villa
authors.affiliation: Unità Operativa Epidemiologia, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy; and.
authors.forename: Dario
authors.initials: D
authors.lastname: Consonni
authors.affiliation: Unità Operativa Ematologia, Ospedale S. Eugenio, Roma, Italy;
authors.forename: Laura
authors.initials: L
authors.lastname: Scaramucci
authors.affiliation: Unità Operativa Ematologia, Ospedale S. Eugenio, Roma, Italy;
authors.forename: Paolo
authors.initials: P
authors.lastname: De Fabritiis
authors.affiliation: Unità Operativa Trasfusionale e Immunologia, Centro Regionale Malattie del Sangue, Castelfranco Veneto, Italy;
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Tagariello
authors.affiliation: Struttura Complessa a Direzione Universitaria Ematologia, Dipartimento di Medicina Traslazionale, Università del Piemonte Orientale Amedeo Avogadro, Novara, Italy;
authors.forename: Gianluca
authors.initials: G
authors.lastname: Gaidano
authors.affiliation: Dipartimento di Terapie Cellulari ed Ematologia, Ospedale San Bortolo, Vicenza, Italy;
authors.forename: Francesco
authors.initials: F
authors.lastname: Rodeghiero
authors.affiliation: Unità Operativa Oncoematologia, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy; Università degli Studi di Milano, Milano, Italy.
authors.forename: Agostino
authors.initials: A
authors.lastname: Cortelezzi
authors.affiliation: Unità Operativa Oncoematologia, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy;
authors.forename: Alberto
authors.initials: A
authors.lastname: Zanella
mesh_terms: D000328:Adult; D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy* / Q000276:immunology* / Q000601:surgery; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology*; D004921:Erythropoietin / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D000069283:Rituximab; D012720:Severity of Illness Index; D013156:Splenectomy; D013256:Steroids / Q000627:therapeutic use*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D013256:Steroids; D004921:Erythropoietin; D000069283:Rituximab