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.

Barcellini, W., Fattizzo, B., Zaninoni, A., Radice, T., Nichele, I., Di Bona, E., et al. (2014). Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia: a GIMEMA study of 308 patients. BLOOD, 124(19), 2930-2936 [10.1182/blood-2014-06-583021].

Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia: a GIMEMA study of 308 patients

De Fabritiis, Paolo;
2014-11-06

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.
6-nov-2014
Pubblicato
Rilevanza internazionale
Articolo
Esperti anonimi
Settore MED/15 - MALATTIE DEL SANGUE
English
Barcellini, W., Fattizzo, B., Zaninoni, A., Radice, T., Nichele, I., Di Bona, E., et al. (2014). Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia: a GIMEMA study of 308 patients. BLOOD, 124(19), 2930-2936 [10.1182/blood-2014-06-583021].
Barcellini, W; Fattizzo, B; Zaninoni, A; Radice, T; Nichele, I; Di Bona, E; Lunghi, M; Tassinari, C; Alfinito, F; Ferrari, A; Leporace, Ap; Niscola, P; Carpenedo, M; Boschetti, C; Revelli, N; Villa, Ma; Consonni, D; Scaramucci, L; De Fabritiis, P; Tagariello, G; Gaidano, G; Rodeghiero, F; Cortelezzi, A; Zanella, A
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/319038
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