Background: Autoimmune hemolytic anemia (AIHA) has rarely been reported in association with human immunodeficiency (HIV) infection and never as a presenting manifestation. Similarly, disseminated intravascular coagulation (DIC) is a very infrequent complication of HIV infection.
Case report: An unusual patient is described who at the time of presentation with severe AIHA was found to be HIV positive. Shortly thereafter, he developed DIC, pulmonary thrombi, and right heart failure that proved fatal, in spite of intensive supportive measures.
Conclusion: Although the etiology of the DIC and pulmonary thrombi could not be established, they are most likely related to aggressive transfusion therapy, with associated intravascular hemolysis.