Predictors of refractoriness to therapy and healthcare resource utilization in 378 patients with primary autoimmune hemolytic anemia from eight Italian reference centers

Abstract

Autoimmune hemolytic anemia (AIHA) is a rare and heterogeneous disease with a presentation/phenotype ranging from mild/compensated to life-threatening. 1,2 Moreover, some cases show refractoriness to/relapse after first and further therapy lines, leading to significant and underestimated healthcare burden. Here, we retrospectively analyzed 378 primary AIHA cases (135 M and 243 F, median age 61 years, range 5-94) from eight Italian hematological centers and fol-

Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting

“…Response to different therapies in 24 cases of mixed AIHA was recently analyzed [23]. Corticosteroids were administered as first line therapy in all cases, 67% needed a second line only, and 33% a further third line; 8% displayed multi-refractoriness, requiring further therapy lines.…”

“…In patients with CAD, transfused blood must be prewarmed by the use of commercial warming coils [1,10]. thrombotic risk has been identified in both wAIHA and cAIHA [23,53,192,193]. In two large studies a thrombotic event was recorded in 11% of patients [23,53].…”

“…thrombotic risk has been identified in both wAIHA and cAIHA [23,53,192,193]. In two large studies a thrombotic event was recorded in 11% of patients [23,53]. Moreover, by using a USA cohort of commercial insurance enrollees (Truven Health MarketScan® Databases) the risk of VTE in AIHA was significantly increased (19 per 1,000 person-years, versus 1.9 in the control group) with an adjusted hazard ratio of 6.30 (95% confidence interval: 4.44-8.94) [194].…”

“…Otherwise, clinicians should refer to national guidelines where available, but most currently recommend that for adults age 40-90, the FRAX ® tool (www.sheffield.ac.uk/FRAX/tool.jsp) be used, supplemented by measurement of bone mineral density if available [196,198,199]. In addition concomitant thrombocytopenia (Evans syndrome) and acute renal failure occur in about 10% and 2% of AIHA patients, respectively [23,53]. Although rare, they may be associated with a fatal outcome, with a HR of 7 and 18, respectively.…”

“…Most, if not all, subjects with chronic CAD have an underlying lymphoproliferative disorder [18,19]. Several studies and clinical experience of the authors attests to the chronic nature of AIHA and the difficulty of obtaining durable treatment-free remissions [11,[20][21][22][23].…”