An infant with γ-globulin-induced hypersensitivity syndrome who developed Evans' syndrome after a second γ-globulin treatment

Abstract

One month after treatment with γ-globulin for Kawasaki disease, an 18-month-old girl developed Evans' syndrome in addition to drug-induced hypersensitivity syndrome (DIHS) after a second γ-globulin treatment. She suffered from hyperbilirubinemia, hemolytic anemia, and thrombocytopenia. The findings and her clinical course involved plasma exchange and treatment with prednisolone, with good results. Peripheral lymphocyte stimulation tests indicated that γ-globulin was the likeliest cause of the DIHS. A real-time polymerase chain reaction test showed the human herpes virus (HHV)-6 genome in peripheral blood. We demonstrated that a primary infection or infection reactivation by the HHV-6 virus was involved in the development of γ-globulin-induced hypersensitivity and Evans' syndrome.

Drug‐induced hypersensitivity syndrome by i.v. immunoglobulin administration for Kawasaki disease

“…One month later, she developed Evans syndrome in addition to DIHS after a second IVIG. 2 These two cases including ours suggest a close relationship between Kawasaki disease and IVIG-induced DIHS. This is probably not a coincidence because DIHS caused by IVIG has not been reported in other underlying diseases.…”

“…Notably, only one similar Kawasaki disease case of IVIG-induced DIHS was previously reported. 2 A 17-month-old boy developed Kawasaki disease and was treated with IVIG (2 g/kg for 1 day) and aspirin, which rapidly improved his rash. However, skin eruption reappeared on his face and trunk on the 8th day after IVIG administration.…”

“…A rapid and robust increase in Ig levels by IVIG administration may trigger the development of immune reconstitution inflammatory syndrome and induce various viral reactivations. 2 Only one case of IVIG-induced DIHS has been reported. 2 An 18-month-old Japanese girl with Kawasaki disease was treated with IVIG and aspirin.…”

“…2 Only one case of IVIG-induced DIHS has been reported. 2 An 18-month-old Japanese girl with Kawasaki disease was treated with IVIG and aspirin. One month later, she developed Evans syndrome in addition to DIHS after a second IVIG.…”

Pediatric drug reaction with eosinophilia and systemic symptoms: A systematic review of the literature

“…DRESS is rare, with an estimated incidence of 1 per 1000 to 1 per 10 000 cases per drug exposure . Reported mortality is estimated at 10%, and long‐term as well as delayed sequelae are documented in a variety of case reports . While comprehensive reviews have been performed in the adult population, an extensive literature review of DRESS in the pediatric population has not been published.…”