Fatal Immune Hemolytic Anemia and Hepatic Failure Associated with a Warm‐Reacting IgM Autoantibody

Abstract

Autoimmune hemolytic anemia (AIHA) caused by warm-reacting IgM autoantibodies is rare. We report a fatal case of primary AIHA with a warm-reacting IgM autoantibody. Recurrent episodes of intravascular hemolysis, unresponsive to all therapy and progressive hepatic dysfunction characterized the patient's clinical course. Despite corticosteroid therapy, splenectomy and multiple blood transfusions, the patient died from liver failure. The IgM autoantibody caused autoagglutination of the patient's red cells at 37 degrees C. Eluates prepared from the patient's red cells agglutinated saline-suspended test cells without the addition of antiglobulin reagent. We propose that warm-reacting IgM antibodies may lead to in vivo autoagglutination and may be associated with hepatic failure.

Membrane autoantibodies in systemic lupus erythematosus: a case of autoimmune hemolytic anemia, antiphospholipid antibodies, and transient acquired activated protein C resistance

“…Mixed‐type AIHA appears to respond to treatment in a manner similar to IgG warm AIHA 10,22 . Case reports of IgM warm AIHA typically describe an aggressive intravascular hemolytic anemia sometimes accompanied by tissue ischemia resulting from agglutination of RBCs in vascular beds 14‐16,23‐25 . In these cases, the disease process was refractory to various combinations of transfusion support, plasma exchange, corticosteroids, cytotoxic agents, or splenectomy, and clinical outcomes were very poor.…”

Hepatic Manifestations in Hematological Disorders

“…Immunoglobulin (Ig)G antibodies (rarely IgM antibodies) generally react with antigens on the RBC surface at body temperature and are thus referred to as “warm agglutinins,” whereas IgM antibodies (rarely IgG type) react with antigens on the RBC surface below body temperature and are thus referred to as “cold agglutinins.” Warm-reacting IgM antibodies may lead to hepatic failure by in vivo autoagglutination [16]. A fatal case with primary AIHA presenting as acute liver failure has been reported [16].…”

“…A fatal case with primary AIHA presenting as acute liver failure has been reported [16]. The patient experienced recurrent episodes of intravascular hemolysis.…”

Severe Autoimmune Hemolytic Anemia in an Infant Caused by Warm-reactive IGM and IGA Autoantibodies

“…In these rare cases, most patients have had a chronic and relapsing course with multiple fatalities reported. [3][4][5][6] The 1 reported exception was a patient with severe warm-reactive IgMmediated AIHA in the setting of primary Sjo¨gren syndrome, who recovered with corticosteroid therapy alone. 7 There are limited reports of warm-reactive IgM-mediated AIHA in children, suggesting prognosis is superior to that seen in adults (Table 1).…”