Clinical Analysis Of 21 Patients With Hemophagocytic Syndrome And Literature Review

Objective:Summarized retrospectively analysis the clinical features of patients with HPS,combined with domestic and foreign literature review and discussion,aimed at improving all levels of various professional clinicians on the recognitiong and diagnostic level of the disease.Methods:In recent five years(2011.1-2015.12),21 patients of HPS were continuously collected from the second hospital of shanxi medical university, summarized their medical records(etiologys,clinical features,laboratory examinations,major treatment strategies and outcomes),explored possible factors that influenced the prognosis of disease.Results:Total 21 patients, 9 cases(42.86%)of men and women in 12 cases(57.14%),the ratio of both men and women was 3:4;The overall age distribution interval for 18-76 years old,the median age was 43.The most common cause of 21 HPS patients was infection(14cases), of which the most common EBV infection(10 cases);lymphoma and of unknown etiology in 2 cases and 5 cases respectively.The major clinical manifestations were:fever and splenomegaly(19 cases), pulmonary infection(16 cases),hepatomegaly(12cases),lymphadenectasis and serous cavity effusion of 11 cases and 10 cases respectively;in addition rare yellow dye, petechiae,ecchymoses and skin rash;a few merger of central nervous system symptoms,cardiac insufficiency,renal insufficiency and gastrointestinal bleeding.Common for laboratory examination: two or three ofhypocytosis(21 cases),20 patients have tested ferritin(SF),of which 19 hyperferritinemia,liver dysfunction(18 cases),hemophagocytosis in bone marrow(17cases),18 patients have tested NK-cell activity,of which 13 with low/absent NK-cell activity;hypertriglyceridemia(10 cases),hypofibrinogenemia and coagulopathy in 14 cases respectively,3 cases with disseminated intravascular coagulation,1 patient detected sCD25 increased significantly.Treatment:active control of the primary disease,the mainly therapeutic strategies were corticosteroids,etoposide and/or cyclosporine;lymphoma patients accepted CHOP chemotherapy(2 cases);4 patients received high-dose methylprednisolone shock therapy,1 case of TTP used in combination with plasma exchange.Outcome,9 cases of patients with early hospital discharge,12 patients died because of aggravation.Among died patients,1 case merged of gastrointestinal bleeding,lung infection and coagulopathy in 10 cases respectively, 3 cases merge of DIC,5 cases of organ failure.Comparison of the basic situations of incipient,which included age,gender, main complications and early laboratory indexes,found that two groups begin with white blood cell count(WBC), platelet count(Plt), hemoglobin(Hb) and serum ferritin(SF) difference were statistically significant(all P<0.05).Conclusion :Infection and tumor were common causes of secondary HPS.Clinical features mainly were fever,hepatomegaly,splenomegaly,lymphadenectasis,the two or three hypocytosis,hyperferritinemia,low or absent NK-cell activity,hemophagocytosis in bone marrow,hypofibrinogenemia.Most of them have liver dysfunction,pulmonary infection and coagulopathy.Early diagnosis and timely treatment may improve the prognosis.Begin with low leukocyte count and platelet count, anemia and ferritin markedly increase may be associated with poor prognosis.The reduced counts of platelet was the independent risk factor of prognosis.Combined with liver dysfunction,coagulopathy, lung infection and viscera function failure usually with worse prognosis.