Characteristics and outcome of warm autoimmune hemolytic anemia in adults: New insights based on a single-center experience with 60 patients.
Warm autoimmune hemolytic anemia (wAIHA) is a rare autoimmune disease with poorly known natural history and management remaining mainly empirical. To better describe the characteristics and outcome of wAIHA in adults, we performed a single-center cohort study of patients diagnosed with wAIIHA from 2001 to 2012 in our center. Sixty patients (50% women) were included, the mean age at the time of wAIHA onset was 54 ± 23 years. wAIHA was considered "primary" for 21 patients (35%) and was associated with an underlying disorder in 39 (65%), including mainly lymphoproliferative disorders and systemic lupus. All patients but two needed treatment and received corticosteroids, with an overall initial response rate of 87%. However, 63% of the patients were corticosteroid-dependent and 56% required at least one second-line treatment including mainly rituximab (n = 19). At the time of analysis, after a mean follow-up of 46 months, 28 patients (47%) were in remission and off treatment and 5 (8%) had died. The presence of an underlying lymphoproliferative disorder was associated with reduced response to corticosteroids and increased need for second-line therapy. In conclusion, in the last decade and compared to a previous series from our center, the rate of secondary wAIHA has increased and the use of rituximab has emerged as the preferred second-line treatment and corticosteroid-sparing strategy; the overall mortality has significantly decreased (8 vs. 18%).
APHP, Henri Mondor Hospital, Department of Internal Medicine, French National Referral Center For Adults 'Immune Cytopenias, Créteil, France.
Mathilde
M
Roumier
Valentine
V
Loustau
Constance
C
Guillaud
Laetitia
L
Languille
Matthieu
M
Mahevas
Mehdi
M
Khellaf
Nicolas
N
Limal
France
F
Noizat-Pirenne
Bertrand
B
Godeau
Marc
M
Michel
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy* / Q000209:etiology* / Q000276:immunology* / Q000401:mortality; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D015331:Cohort Studies; D018572:Disease-Free Survival; D017707:Erythrocyte Transfusion; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000401:mortality; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000401:mortality; D008297:Male; D008875:Middle Aged; D000069283:Rituximab; D013156:Splenectomy; D016896:Treatment Outcome; D055815:Young Adult
D058846:Antibodies, Monoclonal, Murine-Derived; D005938:Glucocorticoids; D006454:Hemoglobins; D000069283:Rituximab