Hemophagocytic lymphohistiocytosis in a rheumatoid arthritis patient treated with infliximab.
ABSTRACT Hemophagocytic lymphohistiocytosis (HLH) is a rare condition with high mortality. We report a case of a 74-year-old woman with rheumatoid arthritis who developed HLH secondary to pyelonephritis due to Escherichia coli infection following infliximab treatment. Bone marrow aspiration showed proliferation of histiocytes with hemophagocytosis. The patient died despite treatment with intravenous antibiotics intravenous methylprednisolone and intravenous immunoglobulin. Cytokine levels were measured and are discussed.
- The American Journal of Medicine 02/1997; 102(1):113-5. · 4.77 Impact Factor
- [show abstract] [hide abstract]
ABSTRACT: 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.American Journal of Hematology 02/2006; 81(1):59-61. · 4.00 Impact Factor
- [show abstract] [hide abstract]
ABSTRACT: Hemophagocytic histiocytic syndrome (HHS) generally occurs in immunocompromised patients and often has a rapidly fatal course. HHS may be cured by treatment of the underlying disorder, especially when it is triggered by an infection. If no cause has been found, no therapy is known and outcome is poor. The aim of this study was to investigate the clinical course and response to intravenous immunoglobulin treatment in renal transplant patients diagnosed with HHS. Thirteen patients who were diagnosed with HHS between 1995 and 2003 were retrospectively assessed. The mean age of HHS patients was 38.6 +/- 10 years (5 women, 8 men). Median time to onset of symptoms after renal transplantation was 15.1 +/- 12.1 months (range 0.5-30 months). The first 2 patients in whom no etiologic factor was found were seen before 1998 and died due to multiorgan failure. HHS was related to an infectious etiology in 6 of 13 patients: tuberculosis (n=3), cytomegalovirus (CMV) infection (n=2), Escherichia coli (E. coli)-associated septicemia (n=1), but HHS was cured by antimicrobial therapy in only 2 of them (1 with tuberculosis, the other with E. coli-associated septicemia). After June 1998, high-dose immunoglobulin (IVIg) therapy was used in 6 patients. HHS was related to an infectious etiology in 2 patients unresponsive to antimicrobial treatment, and of unknown etiology in 4 patients. All of them completely recovered. Before 1998, 2 patients unresponsive to antimicrobial therapy (1 with tuberculosis, the other with CMV) died. They were not given IVIg. We concluded that when HHS does not respond to treatment of the underlying infection, or is of unknown etiology in immunocompromised patients, high-dose IVIg therapy should be administered.Journal of nephrology 19(3):322-6. · 2.02 Impact Factor