In addition to the treatment they received for the underlying causes, patients received therapy for HLH consisting of corticosteroids, immunosuppressive drugs, and intravenous immunoglobulin. Twenty-six patients (25.2%) recovered after treatment, and 19 of them achieved long-term remission during follow-up. Seventy-seven patients (74.8%) died because of tumor, sepsis, multiple organ failure, or HLH-related organ hemorrhage and coagulopathy. The deceased patients were more likely to be older at disease onset, male, and to present with splenomegaly and thrombocytopenia, compared to the survivors. Treatment for the underlying diseases combined with corticosteroids, immunosuppressive agents, and immunoglobulin therapy may improve the prognosis of HLH. More attention should be paid to high-risk patients to prevent the development of serious complications associated with HLH.
Keywords: AOSD = adult-onset Still disease; CMV = cytomegalovirus; CNS = central nervous system; DIC = disseminated intravascular coagulation; EBV = Epstein-Barr virus; HLH = hemophagocytic lymphohistiocytosis; IVIg = intravenous immunoglobulin; MAS = macrophage-activation syndrome; NK = natural killer; RA = rheumatoid arthritis; SLE = systemic lupus erythematosus; autoimmune diseases; clinical manifestation; hemophagocytic lymphohistiocytosis; lymphoma; prognosis; risk factor
Document Type: Research Article
Affiliations: From the Department of Rheumatology and Clinical Immunology (JL, QW, W. Zheng, XT), Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing; and Renal Department of Internal Medicine (JM), Hematology Department of Internal Medicine (W. Zhang), and Pathology Department (WW), Peking Union Medical College Hospital, Beijing, China.
Publication date: March 1, 2014