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MODERN RHEUMATOLOGY Vol.14 No.4

>MR14-4

An infant with γ-globulin-induced hypersensitivity syndrome who developed Evans' syndrome after a second γ-globulin treatment
Takako Miyamae1, Rumiko Kurosawa1, Masaaki Mori1, Yukoh Aihara1, Michiko Aihara2 and Shumpei Yokota1

(1) Department of Pediatrics, Yokohama City University School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan
(2) Department of Dermatology, Yokohama City University School of Medicine, Yokohama, Japan

Received: 27 August 2003 Accepted: 15 March 2004

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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.
 
Key words
Autoimmune hemolytic anemia and thrombocytopenia - γ-Globulin - Hypersensitivity syndrome (DIHS) - Intrahepatic cholestasis - Kawasaki disease
 
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