Characteristics and outcome of warm autoimmune hemolytic anemia in adults: New insights based on a single‐center experience with 60 patients

Abstract

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 6 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 5 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%).Am. J. Hematol. 89:E150-E155,

Autoimmune Hemolytic Anemia Triggered by Infection with Human Parvovirus B19 after Total Abdominal Colectomy for Ulcerative Colitis

“…Roumier et al (19) found that only 3.3% of patients with common AIHA did not require treatment, and that approximately half of healthy adults with warm AIHA that developed following an initial cytomegalovirus infection attained spontaneous remission in the follow-up (20). In AIHA induced by a viral infection, the rate of spontaneous remission with decreased infection is likely to be high.…”

Autoimmune Hemolytic Anemia Confers an Independent Risk Factor for Thrombosis: Retrospective Cohort Study Using the “STRIDE” Database

“…Prior studies have been smaller in size or descriptive [5,26,27], done in the context of specific hyper coagulable states [28,29], or with a design that excluded secondary AIHA patients or lacked anon-AIHA cohort matched for relevant AIHA risk factors [14][15][16][17]19,20].…”

“…Depending on the thermal range at which the auto antibodies are most active, AIHA is classified as warm (wAIHA), cold (cold agglutinin disease, or CAD), or mixed. 50-60% of AIHA cases are believed to be secondary [3][4][5]. Major risk factors for secondary AIHA include malignancy [6], viral and mycoplasma infections [7,8], and rheumatologic disorders [9].…”

Autoimmune hemolytic anemia: transfusion challenges and solutions

“…Although the mean number of packed RBC units was 3.9 at the AIHA onset and 10 during clinical follow-up, no transfusion-related adverse events were observed. 34 Das et al transfused 14 WAIHA patients with severe anemia reflected by hemoglobin values ranging from 3.3 to 6.5 g/dL, and reported that only one individual presented chills with no other adverse effects of transfusion observed. 28 The association of WAIHA with other illnesses has also to be considered before indicating transfusion because patients with cardiac disease show less tolerance to acute anemia thus RBC transfusions must be performed to maintain the hemoglobin at a clinically acceptable level until other measures to stop the hemolysis can take effect.…”

“…These symptoms generally occur in patients with very profound anemia showing hemoglobin levels below 5 g/dL (Table 4). 1,6,17,18,28,34 In mild or moderate anemias (Hb>8 g/ dL) RBC transfusion is rarely necessary or desirable, as symptoms generally are controlled with appropriated therapy. For patients showing hemoglobin level between 5 and 8 g/dL the clinical decision for transfusion is more difficult.…”