Retrospective analysis of patients diagnosed with hemophagocytic lymphohistiocytosis in Van yüzüncü Yil University Faculty of Medicine, department of pediatric hematology

Aim. In this study, it was aimed to investigate the clinical and laboratory findings of patients diagnosed with hemophagocytic lymphohistiocytosis (HLH) in Van Yüzüncü Yıl University Department of Pediatrics and Diseases, and to determine the factors that may affect patients' treatment response, prognosis and survival. Method. The study included patients between 0-18 years old who were diagnosed with HLH between January 1, 2011 and November 30, 2019 at the Pediatric Hematology Outpatient Clinic of Van Dursun Odabaşı Medical Center Hospital. A p value of less than 0.05 was considered significant. Results. 34.1% (n = 14) of cases were PHL, 65.9% (n = 27) of SHL, 54.7% (n = 22) of men, mean age was 85.8 ± 67.0 months . While the gender distribution of PHL and SHL patients is not statistically different, the age of PHL patients is statistically significantly lower (p = 0.001). 48.8% (n = 20) of the patients are parents and relatives, 7.3% (n = 3) have a history of ex-sibling. The frequency of parental consanguinity in patients in the PHL group is statistically significantly higher (p = 0.037). While 80.5% (n = 33) of the patients responded to treatment, 19.5% (n = 8) were ex. The frequency of response to treatment does not change according to the disease type (p = 0.411). At the start of treatment, 82.9% (n = 34) of the patients had fever, 75.6% (n = 31) hepatomegaly, 73.2% (n = 30) splenomegaly, 26.8% (n = 11) LAP, 24.4% (n = 10) rash and neurological findings were detected. Fever decreased significantly in the first week of treatment (p> 0.05). The frequency of symptoms does not differ in patients with PHL and SHL. In general, in the first week of treatment in patients with HLH and SHL, Plt, Na, Ca, total protein and albumin values increased statistically significantly, CRP, LDH, AST, triglyceride and ferritin values decreased significantly (p <0.05). Compared with pretreatment in PHL patients, Hb value increased statistically significantly (p = 0.041) and CRP value decreased statistically significantly in the first week of treatment (p = 0.016). While the cause of 42.9% (n = 6) of PHL patients is unknown, 28.6% (n = 4) perforin gene mutation, 21.4% (n = 3) Chediak-Higashi Syndrome, 7%, 1 (n = 1) is caused by Griscelli Syndrome. The most common 3 reasons in SHL are respectively; 18.5% (n = 5) brucellosis, 14.8% (n = 4) EBV, 7.4% (n = 2) ALL. Conclusion. Although acute phase reactants are high at the time of diagnosis in HLH, both fever decreases and the level of acute phase reactants decreases in the first week of treatment. Also, platelet level improves. Early diagnosis and treatment of HLH patients is very important and the acute clinic is rapidly improving. With comprehensive and multicenter studies, it may be useful to determine the variables that may affect the prognosis of HLH and to regulate the treatment of the disease accordingly.