Success with infliximab in treating refractory hemophagocytic lymphohistiocytosis.
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder characterized by fever, pancytopenia, hepatosplenomegaly, liver dysfunction, and hemophagocytosis. A 29-year-old woman, diagnosed with systemic lupus erythematosus in 1996, developed HLH in early June 2002. HLH remained refractory during 1.5 months of treatment including corticosteroid, cyclosporine, plasma exchange, vincristine, and etoposide. Infliximab (5 mg/kg/day) was then administered twice. After the second administration, the patient attained remission. Because HLH itself is not a neoplasm but an uncontrolled immune reaction, blocking cytokines involved in the reaction should have therapeutic potentials. For HLH patients not responding to conventional therapy, anticytokine treatment with infliximab may represent one of promising options.
Henzan
Tomoko
T
Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan.
Nagafuji
Koji
K
Tsukamoto
Hiroshi
H
Miyamoto
Toshihiro
T
Gondo
Hisashi
H
Imashuku
Shinsaku
S
Harada
Mine
M
D000328:Adult; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D018501:Antirheumatic Agents / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D000069285:Infliximab; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D010951:Plasma Exchange; D012074:Remission Induction
D000911:Antibodies, Monoclonal; D018501:Antirheumatic Agents; D000069285:Infliximab