Clinical analysis of 45 cases of hemophagocytic syndrome
- VernacularTitle:噬血细胞综合征45例临床分析
- Author:
Jie MA
;
Wenjie ZHENG
;
Xuan ZHANG
;
Fulin TANG
- Publication Type:Journal Article
- Keywords:
hemophagocytic syndrome;
underlying diseases;
treatment;
prognosis
- From:
Basic & Clinical Medicine
2010;30(2):189-193
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analize the clinical features of patients with hemophagocytic syndrome (HI'S). Methods The underlying diseases, clinical characteristics, laboratory findings, responses to therapy as well as their outcomes of 45 patients with HPS were analyzed retrospectively. Results The underlying diseases included virus infection (n = 9) , tuberculosis (n =2), rheumatic fever (n =4) , malignancies (n =22) , unknown diseases (n =8). HPS was clinically characterized by high fever(93. 3% ) , hepatomegaly (77. 8% ) , splenomegaly(80% ) , lymphadenopathy (55. 6% ) , skin rash(24.4% ), gastrointestinal hemorrhage(22. 2% ) , renal (35. 6% ) and central nervous systern involvement( 15. 6% ) , five patients presented with disseminated intravascular coagulation( DIC) (11. 1% ). Laboratory data mainly manifested with cytopenia ( 100% ) , liver dysfunction ( 77. 8% ) , hypofibrinogenemia (62. 8% ), hypertriglyceridemia (71. 1% ) , serum ferritin >500 p,g/L(87. 5% ) , low NK-cell activity(92. 9% ) and hemophagocytosis in bone marrow (100% ). Based on treating underlying diseases and use of corticosteroids and immunosuppressive agents in combination with intravenous immunoglobulins(IVIG) therapy, 14 patients recovered , 19 patients died in the hospital, and other 12 cases give up treatment be cause of exacerbation of the disease.Conclusion There are various underlying diseases and clinical manifestations for HPS. HPS is always extremely dangerous with high mortality. Treatment of underlying diseases, corticosteroids, immunodepressant and IVIG may improve the prognosis of HPS.
