A case report of successful treatment with plasma exchange for hemophagocytic syndrome associated with severe systemic juvenile idiopathic arthritis in an infant girl.
An infantile case of hemophagocytic syndrome (HPS) with systemic juvenile idiopathic arthritis (s-JIA), refractory to methylprednisolone pulse therapy and cyclosporine A administration, was successfully treated by plasma exchange. The patient was a one-year-old Japanese girl who had developed recurrent steroid-dependent signs, including fever, skin eruption, and hepatopathy, while in France, where she had been diagnosed as having s-JIA at eight months of age. As a high fever and rheumatoid rash were evident on arrival at our hospital, she was admitted and given intravenous methylprednisolone pulse therapy and cyclosporine A. She developed pancytopenia with a generalized clonic seizure, high fever, and liver dysfunction after her cytomegalovirus (CMV) titer became positive during the course of treatment; therefore, she was treated with ganciclovir. She was subsequently diagnosed as having HPS complicating s-JIA from the findings of a bone marrow aspirate. At this time, her blood examination data including a high level of C-reactive protein and hyperferritinemia, suggested that her s-JIA was very active, and the pancytopenia continued after her CMV titer became negative. Therefore, CMV infection against a background of active s-JIA could have triggered the HPS in this case. Because the HPS was resistant to an immunosuppressive regime of methylprednisolone pulse therapy and cyclosporine A, plasma exchange therapy was started. After three sessions of this therapy, the patient's symptoms and laboratory data were markedly improved. Our experience suggests that plasma exchange should be considered as a therapeutic tool for HPS refractory to conventional therapy.
Nakakura
Hyogo
H
Department of Pediatrics, Osaka Medical College, Osaka, Japan.
Ashida
Akira
A
Matsumura
Hideki
H
Murata
Takuji
T
Nagatoya
Katsuyuki
K
Shibahara
Nobuhisa
N
Inoue
Toru
T
Tamai
Hiroshi
H
D018501:Antirheumatic Agents / Q000627:therapeutic use; D001171:Arthritis, Juvenile / Q000150:complications*; D016572:Cyclosporine / Q000627:therapeutic use; D003586:Cytomegalovirus Infections / Q000150:complications; D005260:Female; D006801:Humans; D007223:Infant; D007564:Japan; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology / Q000628:therapy*; D008775:Methylprednisolone / Q000627:therapeutic use; D010198:Pancytopenia / Q000150:complications; D010951:Plasma Exchange / Q000379:methods*; D012720:Severity of Illness Index; D016896:Treatment Outcome
D018501:Antirheumatic Agents; D016572:Cyclosporine; D008775:Methylprednisolone