Clinical analysis of 12 cases with hemophagocytic syn drome / 中华风湿病学杂志

ABSTRACT

Objective To enhance the understanding of hemophagocytic syndrome(HPS)by analyzing the clinical manifestations, diagnosis and therapy. Methods The clinical data of 12 patients with HPS were retrospectively collected in the People's Hospital of Jiangsu Province from 2000 to 2007. The relevant literature were reviewed. Results Twelve patients were diagnosed as secondary hemophagocytic syndrome most secondary to virus and bacteria infection. Some patients condition was associated with systemic lupus erythematosus or histiocytic necrotizing lympheadenitis. All of the 12 patients had high fever, abnormal liver function and showed a decrease in the number of blood cells in a short time. After antivirus and antibiotic treatment, 11 patients'condition were improved and 1 patient died. Conclusion Hemophagocytic syndrome is not a common clinical condition but with poor prognosis. When patient presents with fever without apparent reasons and pancytopenia, bone marrow examination should be done and sometimes repeated bone marrow examinations are needed. The diagnosis of secondary haemophagocytic syndrome needs multidisciplineary cooperation. Aggressive diagnostic procedures are needed to clarify the diagnosis and prompt treatments are warranted to improve prognosis.