The clinical course, labrotary findings and therapeutic responses of patients with hemophagocytic lymphohistiocytosis in the city of Gaziantep

OBJECTIVE: This study investigated patients with HLH were followed between January 2009- June 2013 for demographic data, clinical and laboratory findings, treatment options, treatment effects on laboratory findings, factors responsible for etiology, and the estimated life span. METHODS:20 patients with primary hemophagocytic syndrome, 18 patients with secondary hemophagocytic syndrome, a total of 38 patients were included in the study. Histiocyte Society criteria for the diagnosis of HLH was used. Patients were retrospectively analyzed. Complete blood count, erythrocyte sedimentation rate, CRP, AST, ALT, albumin, total bilirubin, direct bilirubin, electrolytes, LDH, TG, fibrinogen, ferritin, culture and serological tests were performed in our hospital, department of Biochemistry and Microbiology laboratory. Mutation analysis studies were performed in Biology and Medical Genetics laboratories of our hospital and Pasteur laboratories in France. SPSS for Windows version 11.5 software package is used for statistical analysis and p <0.05 was considered statistically significant. RESULTS: Age of the patients at diagnosis ranged from 3 to 168 months. Death from Primary HLH was found to be even more between relatives and brothers. The most common symptoms were fever, splenomegaly, hemophagocytosis, and the presence of hyperfibrinemia. Mutations detected in patients with primary HLH were PRF1(30%), STX11 (15%), Munc13-4 (5%), respectively. CNS involvement was detected in 35% of patients with primary HLH, and 5 of them died. Hypofibrinojenemia was diagnosed in 70% (n:14) of primary HLH patients and 38,8% (n:7) of secondry HLH patients (p:0,05). 10 out of 20 primary HLH patients had bilirubinemia.The mean total bilirubin level was 3±0,6 mg/dl and the mean direct bilirubin level was 2,3±0,5 mg/dl at the time of diagnosis.7 out of 10 patients died. 18 patients in secondry HLH group did not have hyperbilirubinemia at the time of diagnosis, their mean total bilirubin level was 0,2±1,7 mg/dl (0,09-0,6), and their mean direct bilirubin level was 0,4±0,08 mg/dl (0,1-1,6). C-reactive protein levels were found to be higher in patients with secondry hemophagocytic sydrome compered to patients with primary hemophagocytic sydrome (p:0,017). In primary group, the expected average life was 619 ± 134 days and survival rate was %40 whereas in secondary group expected average life was 1084 ± 87 days and the survival rate was 88.9%. CONCLUSIONS:Total bilirubin and direct bilirubin levels in patients with primary hemophagocytic syndrome was statistically higher than patients with secondary HLH in the study. Relationship between bilirubin levels and mortality were not statistically significant. Life expectancy and the survival rate was higher in patients with secondary HLH.