Clinical features and outcomes of autoimmune hemolytic anemia: a retrospective analysis of 32 cases

Abstract

BackgroundThere has been no report on the clinical features or natural history of autoimmune hemolytic anemia (AIHA) in the Korean adult population. This study retrospectively analyzed the clinical characteristics and long-term outcomes of AIHA in the Korean adults.MethodsPatients newly diagnosed with AIHA between January 1994 and December 2010 at Chungnam National University Hospital were enrolled. Patient characteristics at diagnosis, response to treatment, and the natural course of the disease were documented.ResultsThirty-two patients (31 females and 1 male) with a median age of 48 years (range, 17-86) were enrolled. Of these, 21.9% were initially 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, 0.5-238), 14 of 25 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, 4.3-11.7), 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).ConclusionPrimary AIHA is rare. Regular, vigilant testing for SLE is required in patients initially diagnosed with AIHA.

Warm autoimmune hemolytic anemia: experience from a single referral center in Mexico City

“…This gender predilection could be attributed to the fact that women are more susceptible to autoimmune diseases. The median age at diagnosis was 36 years, which is slower than the previous reports (i.e., 50 yrs) [1617]. This discrepancy can be explained by the fact that our institution is a referral center for SLE and APLS, which are diseases that mostly affect women of childbearing age.…”

Splenic infarction, warm autoimmune hemolytic anemia and antiphospholipid antibodies associated with systemic lupus erythematosus

“…In patients with SLE and concomitant Coombs-positive AIHA, anticardiolipin antibodies (aCL) are found more frequently and at higher titers than in SLE patients without AIHA [2,3].…”

Serum BAFF and APRIL levels in patients with autoimmune hemolytic anemia and their clinical significance

“…The disease is greatly heterogeneous, ranging from cases developing gradually or fully compensated to patients with fulminant presentation and rapid onset of life-threatening anemia [4]. Despite a long history for this disorder, management of AIHA is still based mainly on empirical data and the results of small, retrospective, and uncontrolled studies [5]. A better understanding of the basic mechanisms underlying the development of AIHA is urgently needed.…”