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: Wilma Barcellini, Bruno Fattizzo, Anna Zaninoni, Tommaso Radice, Ilaria Nichele, Eros Di Bona, Monia Lunghi, Cristina Tassinari, Fiorella Alfinito, Antonella Ferrari, Anna Paola Leporace, Pasquale Niscola, Monica Carpenedo, Carla Boschetti, Nicoletta Revelli, Maria Antonietta Villa, Dario Consonni, Laura Scaramucci, Paolo De Fabritiis, Giuseppe Tagariello, Gianluca Gaidano, Francesco Rodeghiero, Agostino Cortelezzi, Alberto Zanella
• Journal: Blood (Blood) Vol. 124 Issue 19 Pg. 2930-6 (Nov 06 2014) ISSN: 1528-0020 [Electronic] United States
• PMID: 25232059 (Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)
• Copyright: © 2014 by The American Society of Hematology.

Chemical References

• Antibodies, Monoclonal, Murine-Derived
• Autoantibodies
• Immunoglobulins, Intravenous
• Immunologic Factors
• Immunosuppressive Agents
• Steroids
• Erythropoietin
• Rituximab

Topics

• Adult
• Aged
• Anemia, Hemolytic, Autoimmune (drug therapy, immunology, surgery)
• Antibodies, Monoclonal, Murine-Derived (therapeutic use)
• Autoantibodies (immunology)
• Erythropoietin (therapeutic use)
• Female
• Humans
• Immunoglobulins, Intravenous (therapeutic use)
• Immunologic Factors (therapeutic use)
• Immunosuppressive Agents (therapeutic use)
• Male
• Middle Aged
• Predictive Value of Tests
• Retrospective Studies
• Rituximab
• Severity of Illness Index
• Splenectomy
• Steroids (therapeutic use)
• Treatment Outcome
• Young Adult