TY - JOUR
T1 - Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia
T2 - A GIMEMA study of 308 Patients
AU - Barcellini, Wilma
AU - Fattizzo, Bruno
AU - Zaninoni, Anna
AU - Radice, Tommaso
AU - Nichele, Ilaria
AU - Di Bona, Eros
AU - Lunghi, Monia
AU - Tassinari, Cristina
AU - Alfinito, Fiorella
AU - Ferrari, Antonella
AU - Leporace, Anna Paola
AU - Niscola, Pasquale
AU - Carpenedo, Monica
AU - Boschetti, Carla
AU - Revelli, Nicoletta
AU - Villa, Maria Antonietta
AU - Consonni, Dario
AU - Scaramucci, Laura
AU - De Fabritiis, Paolo
AU - Tagariello, Giuseppe
AU - Gaidano, Gianluca
AU - Rodeghiero, Francesco
AU - Cortelezzi, Agostino
AU - Zanella, Alberto
N1 - Publisher Copyright:
© 2014 by The American Society of Hematology.
PY - 2014/11/6
Y1 - 2014/11/6
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=84909592643&partnerID=8YFLogxK
U2 - 10.1182/blood-2014-06-583021
DO - 10.1182/blood-2014-06-583021
M3 - Article
SN - 0006-4971
VL - 124
SP - 2930
EP - 2936
JO - Blood
JF - Blood
IS - 19
ER -