Warm autoimmune hemolytic anemia (wAIHA) is the most common form of AIHA, with corticosteroids in first-line treatment resulting in a 60-80% response rate. Atypical wAIHA and IgG plus complement mediated disease have a higher treatment failure rate and higher recurrence rate. We report a case of severe wAIHA secondary to Waldenström macroglobulinemia with life threatening intravascular hemolysi...

Open heart surgery is rarely performed on patients with heart disease complicated by autoimmune hemolytic anemia (AIHA) with the aid of an artificial heart-lung machine. We successfully performed radical open heart surgery on an atrial septal defect (ASD) patient with the complication of AIHA.

Case 1. Robert Davies, a 50-year-old man, presents with tiredness and lethargy. Examination demonstrates pallor and jaundice. Investigations reveal anemia that is consistent with warm autoimmune hemolytic anemia (AIHA; Figure 1). He has no evidence of any other autoimmune disease or chronic lymphocytic leukemia (CLL) and is not taking any medications; these factors suggest idiopathic AIHA. How ...

In autoimmune hemolytic anemia (AIHA), circulating red blood cells (RBCs) opsonized with autoantibody are recognized by macrophage Fc g and complement receptors. This triggers phagocytosis and elimination of RBCs from the circulation by splenic macrophages. We recently found that CD47 on unopsonized RBCs binds macrophage signal regulatory protein a (SIRP a ), generating a negative signal that p...

The association of systemic lupus erythematosus (SLE) with autoimmune hemolytic anemia (AIHA) is a known phenomenon. In many cases of AIHA, no autoantibody specificity is present. The patient’s serum reacts with all of the red blood cell (RBC) samples tested and the autoantibody appears to have broad specificity in the Rh blood group system. Occasionally, RBC autoantibodies demonstrate apparent...

The association of systemic lupus erythematosus (SLE) with autoimmune hemolytic anemia (AIHA) is a known phenomenon. In many cases of AIHA, no autoantibody specificity is present. The patient’s serum reacts with all of the red blood cell (RBC) samples tested and the autoantibody appears to have broad specificity in the Rh blood group system. Occasionally, RBC autoantibodies demonstrate apparent...

The association of systemic lupus erythematosus (SLE) with autoimmune hemolytic anemia (AIHA) is a known phenomenon. In many cases of AIHA, no autoantibody specificity is present. The patient’s serum reacts with all of the red blood cell (RBC) samples tested and the autoantibody appears to have broad specificity in the Rh blood group system. Occasionally, RBC autoantibodies demonstrate apparent...

The association of systemic lupus erythematosus (SLE) with autoimmune hemolytic anemia (AIHA) is a known phenomenon. In many cases of AIHA, no autoantibody specificity is present. The patient’s serum reacts with all of the red blood cell (RBC) samples tested and the autoantibody appears to have broad specificity in the Rh blood group system. Occasionally, RBC autoantibodies demonstrate apparent...

The glycoprotein CD47 (integrin-associated protein, IAP) is present on the surface of virtually all cells, including red blood cells (RBCs). CD47 acts like a marker of self by ligating the macrophage inhibitory receptor signal regulatory protein alpha (SIRPalpha). In this manner mild reactivity of wild-type RBCs with macrophage phagocytic receptors is tolerated, whereas otherwise identical CD47...

Splenic infarction is most commonly caused by cardiovascular thromboembolism; however, splenic infarction can also occur in hematologic diseases, including sickle cell disease, hereditary spherocytosis, chronic myeloproliferative disease, leukemia, and lymphoma. Although 10% of splenic infarction is caused by hematologic diseases, it seldom accompanies autoimmune hemolytic anemia (AIHA). We rep...