Background There has been no report around the clinical features or

Background There has been no report around the clinical features or natural history of autoimmune hemolytic anemia (AIHA) in the Korean adult populace. diagnosed with secondary AIHA. Thirteen patients (40.6%) were initially diagnosed with Evans’ syndrome. Of the 29 patients who were placed on therapy 27 (93.1%) showed a partial response or better. Nevertheless 1 year after initiating treatment 80 of the patients were still treatment-dependent. During follow-up (median length 14 months; range 0.5 14 of 25 SRT1720 HCl patients (56.0%) who were initially diagnosed with primary warm antibody AIHA were found to have systemic lupus erythematosus (SLE). Median time to conversion to SLE was 8.0 months (95% CI SRT1720 HCl 4.3 and the probabilities of conversion at 12 and 24 months were 63% and 91% respectively. Younger age (<60 years) and a positive fluorescent anti-nuclear antibody test were associated with a higher probability of SLE conversion (P=0.01 and P<0.001 respectively). Conclusion Primary AIHA is Rabbit Polyclonal to MASTL. usually rare. Regular vigilant testing for SLE is required in patients initially diagnosed with AIHA. Keywords: Autoimmune hemolytic anemia Evans’ syndrome Systemic lupus erythematosus Thrombosis INTRODUCTION Autoimmune hemolytic anemia (AIHA) is usually defined as the increased destruction of red blood cells (RBCs) in the presence of anti-RBC autoantibodies [1]. AIHA is usually a relatively uncommon cause of anemia. Recent population-based studies have calculated the incidence of AIHA to be 0.8/100 0 [2] and its prevalence to be 17/100 0 [3]. AIHA may be primary (idiopathic) or secondary to various diseases including systemic autoimmune disorders [4-6] malignancies [7] and infections [8 9 AIHA can also be induced by certain drugs [10 11 This disorder is usually heterogeneous with respect to the type (warm or cold) of antibodies involved. In spite of a long history of SRT1720 HCl this disorder management of AIHA is still mainly based on empirical data and on the results of small retrospective uncontrolled studies. Therapies for AIHA have been reviewed by several experts [12-15] but treatment guidelines have not yet been established. The current recommendations for the diagnosis and management of this disorder originate from Western Europe and North America where the epidemiology of hematologic disorders may be different from that in the Orient. Although a few studies have SRT1720 HCl described the clinical characteristics of AIHA in the Asian populations [11 16 information from Asian regions is still limited. Furthermore there has been no report around the clinical features or natural history of AIHA in the Korean adults. In the SRT1720 HCl present study we retrospectively analyzed clinical characteristics and outcomes of patients with AIHA in our institute. MATERIALS AND METHODS 1 Patients Patients who were consecutively diagnosed with AIHA based on positive results to either Coombs’ test or cold agglutinin assay at Chungnam National University Hospital between January 1994 and December 2010 were enrolled. All patients were Koreans. Patients with drug-induced hemolytic anemia were excluded. All patients underwent the following laboratory investigations: CBC with reticulocyte counts peripheral blood smear chemistry (including lactate dehydrogenase [LDH] and direct and indirect bilirubins) urine analysis serum haptoglobin plasma hemoglobin direct and indirect Coombs’ assessments and cold agglutinin assay. Screening assessments for SLE including fluorescent anti-nuclear antibody (FANA) complement-3 (C3) and -4 (C4) assessments were also performed. Patients who were positive for FANA underwent additional studies for autoantibodies such as anti-double strand (ds) DNA antibody and anti-Smith antibody. Lupus anticoagulants (LA) and anti-cardiolipin antibodies (aCL) were examined. Bone marrow studies were performed to rule out lymphoproliferative disorders. SLE was diagnosed according to the American College of Rheumatology revised classification criteria for SLE [21]. Patients fulfilling only 3 of the revised classification criteria for SLE from the American College of Rheumatology were defined as having “incomplete” SLE [22]. Evans’ syndrome was diagnosed if the patient tested positive for hemolytic anemia by the Coomb’s test and for idiopathic thrombocytopenic.