IgM-mediated Warm Autoimmune Hemolytic Anemia: An Autopsy Report

Abstract

A 79-year-old man with Sjögren's syndrome and systemic lupus erythematosus developed acute impaired consciousness and hemolytic anemia. The patient's red blood cells agglutinated spontaneously at 25-37℃. The treatment of red blood cells with 2-mercaptoethanol resulted in the loss of spontaneous agglutination. A diagnosis of IgM-mediated warm autoimmune hemolytic anemia was made. The patient received steroid pulse and plasma exchange therapies. Rituximab was also administered. However, the patient died from multiple organ failure at six days from the symptom onset. The clinical progress of the patient and autopsy findings suggested that complement activation might have been associated with the pathology.

A single dose of eculizumab terminated life‐threatening haemolysis in idiopathic IgM‐mediated warm autoimmune haemolytic anaemia: A case report

“…The life‐threatening nature of haemolysis in our patient is consistent with an IgM‐wAIHA 10–14 . Neurological complications, fever as well as fatal outcomes were reported in several patients with IgM‐wAIHA 10–14 .…”

“…[10][11][12][13][14] Neurological complications, fever as well as fatal outcomes were reported in several patients with IgM-wAIHA. [10][11][12][13][14] Neurological complications might represent vascular occlusion by large RBC agglutinates 15 Or thromboembolic phenomenon that are known to complicate haemolysis. 5 Non-complement fixing IgM autoantibodies were reported to cause AIHA in children, 16 which may again point to a physical role for the agglutinates in the pathophysiology of disease manifestations.…”

“…4 The life-threatening nature of haemolysis in our patient is consistent with an IgM-wAIHA. [10][11][12][13][14] Neurological complications, fever as well as fatal outcomes were reported in several patients with IgM-wAIHA. [10][11][12][13][14] Neurological complications might represent vascular occlusion by large RBC agglutinates 15 Or thromboembolic phenomenon that are known to complicate haemolysis.…”

Multiorgan failure in a fatal case of autoimmune hemolytic anemia

“…4 In another instance, also resulting from IgM AIHA, liver failure was attributed to cholestasis and bile plugs. 5 In this case, the spleen demonstrated extensive extramedullary hematopoiesis primarily involving the red pulp and sinusoids (Figure 2(A)-(C)), and pathology showed no evidence of lymphoma. The liver biopsy demonstrated extramedullary hematopoiesis in a background of cholestatic hepatitis, atrophic bile ducts, mild centrilobular degeneration, and iron accumulation (Figure 2(D)-(F)).…”