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 followedup for a median of 33 months (range12-372); 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 incidenceofrelapse was increasedin 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.

Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia : a GIMEMA study of 308 Patients / W. Barcellini, B. Fattizzo, A. Zaninoni, T. Radice, I. Nichele, E. Di Bona, M. Lunghi, C. Tassinari, F. Alfinito, A. Ferrari, A.P. Leporace, P. Niscola, M. Carpenedo, C. Boschetti, N. Revelli, M.A. Villa, D. Consonni, L. Scaramucci, P. De Fabritiis, G. Tagariello, G. Gaidano, F. Rodeghiero, A. Cortelezzi, A. Zanella. - In: BLOOD. - ISSN 0006-4971. - 124:19(2014 Nov), pp. 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

B. Fattizzo
Secondo
;
T. Radice;A. Cortelezzi
Penultimo
;
2014-11

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 followedup for a median of 33 months (range12-372); 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 incidenceofrelapse was increasedin 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.
Adult; Aged; Anemia, Hemolytic, Autoimmune; Antibodies, Monoclonal, Murine-Derived; Autoantibodies; Erythropoietin; Female; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Immunosuppressive Agents; Male; Middle Aged; Predictive Value of Tests; Retrospective Studies; Severity of Illness Index; Splenectomy; Steroids; Treatment Outcome; Young Adult; Hematology; Biochemistry; Cell Biology; Immunology
Settore MED/15 - Malattie del Sangue
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/257685
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