Intravenous immune globulin therapy for Stevens-Johnson syndrome/toxic epidermal necrolysis complicated by hemolysis leading to pigment nephropathy and hemodialysis.

BACKGROUND: Intravenous immune globulin (IVIG) is generally thought to be of relatively low risk for adverse events and some experts consider this to be the best treatment for Stevens-Johnson syndrome/toxic epidermal necrolysis. OBJECTIVE: We evaluated the underlying cause of anemia and renal failure in 2 consecutive patients being treated with IVIG for Stevens-Johnson syndrome/toxic epidermal necrolysis. METHODS: This is a retrospective chart review. RESULTS: We present 2 patients with Stevens-Johnson syndrome/toxic epidermal necrolysis and severe hemolysis requiring blood transfusion who subsequently developed pigment nephropathy necessitating hemodialysis after treatment with IVIG. Both patients had antibodies to their ABO blood type detected in the eluate from their red blood cell membrane. LIMITATIONS: This is a retrospective review with only 2 cases. CONCLUSIONS: We propose that IVIG-associated hemolysis is an adverse reaction that may not be as rare as once thought, presenting as a mild decrease in hemoglobin and hematocrit. Antibodies to blood type A and B are given as part of pooled immune globulin and are considered to be the cause of hemolysis. More severe anemia requiring transfusion is less common, and the breakdown products produced by hemolysis can lead to pigment nephropathy and renal failure. We present methods by which this severe complication can be anticipated and managed more effectively.