Intravenous Immunoglobulin Associated With Hemolytic Anemia in Kawasaki Disease

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Kawasaki is a rare hematologic disease that occurs in fewer than 20,000 patients in the United States annually.
Kawasaki is a rare hematologic disease that occurs in fewer than 20,000 patients in the United States annually.

Patients with Kawasaki disease receiving high-dose intravenous immunoglobulin are at greater risk for hemolysis, according to results published recently in Transfusion.

Kawasaki disease is very rare and is characterized by inflammation of some blood vessels. It occurs in fewer than 20,000 patients in the United States each year, and it primarily affects young children. With an unknown etiology, Kawasaki disease can cause rashes, glandular swelling, and possibly damage to the heart.

Though hemolysis is a known potential side effect in adult patients undergoing therapy with intravenous immunoglobulin, this study assessed its frequency in a pediatric population.

Researchers conducted an 8-year, single-center, retrospective cohort study on 419 patients with Kawasaki disease, 123 of whom underwent pre- and post-treatment complete blood counts, which allowed for determination of anemia. Hemolytic anemia was defined as a decrease in hemoglobin greater than 1 g/dL after therapy with intravenous immunoglobulin. Immunohematologic or biochemical studies supported analysis of hemolysis.

Overall, 15% of patients experienced hemolysis and 85% did not. Patients who experienced hemolysis were more likely to have complete Kawasaki disease than incomplete Kawasaki disease (65% vs 39%; P =.04). They were also more likely to have refractory Kawasaki disease than non-refractory Kawasaki disease (78% vs 16%; P <.001).

Patients who received the higher dose of 4 g/kg of immunoglobulin were more likely to experience hemolysis than patients who received 2 g/kg (89% vs 35%; P <.001). Additionally, the overwhelming majority of patients who experienced hemolysis had non-O blood types (94%), positive direct antiglobulin tests (89%), and positive eluates (71%). Most patients who experienced hemolysis needed a red blood cell transfusion.

Patients who undergo intravenous immunoglobulin therapy to treat Kawasaki disease should be monitored for hemolysis, the authors concluded.

Reference

1. Nolan BE, Wang Y, Pary PP, Luban NL, Wong EC, Ronis T. High-dose intravenous immunoglobulin is strongly associated with hemolytic anemia in patients with Kawasaki disease [published online September 28, 2018]. Transfusion. doi: 10.1111/trf.14879

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