A Case of Fatal Pulmonary Embolism in a Patient With Warm Autoimmune Hemolytic Anemia: An Under-appreciated Thrombotic Risk

Abstract

Autoimmune hemolytic anemia (AIHA) is a rare disorder in which the life span of red blood cells (RBCs) is prematurely decreased by hemolysis by either IgG or IgM auto-antibodies directed against red cell membrane antigens. Many factors are known to trigger this auto-antibody production. Symptoms are non-specific and vary according to the degree of anemia. Although considerable advances have been made in understanding the pathogenesis and securing the diagnosis, progress in management has been slow with no established therapeutic guidelines despite the input of many investigators. Lymphoproliferative disorders (LPDs) and venous thromboembolism (VTE) remain the two most clinically significant associated conditions. VTE is a more serious, acute and often lethal complication of AIHA. There are many mechanisms described for the prothrombotic state in patients with AIHA. Despite the thrombotic implications, risk of VTE is often overlooked and still lacking are standardized risk-stratification criteria and evidence-based thromboprophylactic strategies appropriate for the level of risk. With this case report, our goal is to raise awareness of this frequently unrecognized disorder and its potential for fatal consequences. We also summarize some of the mechanisms involved with the goal of stimulating a clinical discussion aimed at appropriately risk stratifying these patients for robust thromboprophylactic strategies.




doi: http://dx.doi.org/10.4021/jh95w