Demographics in patients with familial white fever, clinical and new hematologic parameters evaluation

Introductıon: Familial Mediterranean Fever (FMF) is a disease of the autoinflammatory periodic fever syndromes, which is common in the Eastern Mediterranean populations, clinically characterized by recurrent fever, polyserositis, erysipelas-like erythema and joint involvement in the form of arthritis, and in which MEFV gene mutations are observed. In our study, demographic, clinical and new hematological parameters were studied in FMF patients. Method: Our study was conducted by evaluating patients over 18 years of age who were followed up with the diagnosis of AAA in the internal medicine outpatient clinics of Tokat Gaziosmanpaşa University Hospital between 01.01.2013-01.01.2023. From our hospital system, 220 patients with ICD diagnosis codes E85.0-E85.9 who met Tel Hashomer diagnostic criteria were included in the AAA patient group. Our study was designed retrospectively. Demographic data including age and gender; clinical symptoms including abdominal pain, fever, chest pain, myalgia, arthritis, erysipelas like rash; new hematologic parameters including RDW, MPV, PDW, neutrophil count, lymphocyte count, platelet count, NLR and PLR rates were recorded. The collected data were evaluated by statistical methods. Results: Of the 220 patients diagnosed with AAA, 56.8% (n=125) were female and 43.2% (n=95) were male. The mean age at first diagnosis was 25.13±12.41 years. Among the clinical findings of the patients during the attack; 55.9% (n=123) had fever, 87.7% (n=193) had abdominal pain, 18.2% (n=40) had chest pain, 36.8% (n=81) had myalgia and arthralgia, 18.2% (n=40) had arthritis, 4.5% (n=10) had erysipelas-like erythema. Patients had a mean of 2.21 symptoms. 45.8% (n=115) had M694V mutation, 16.3% (n=41) had R202Q mutation, 13.1% (n=33) had M680I mutation, 12.3% (n=31) had V726A mutation and 7.1% (n=18) had E148Q mutation There was a significant increase in neutrophil counts, NLR ratio, PLR ratio and a significant decrease in lymphocyte counts in patients in the attack period compared to patients in the non-attack period. The number of symptoms was correlated with neutrophil count and NLR among hematologic parameters. vii Conclusıon: Consistent with the literature, male gender was more common and the most common clinical findings were fever and abdominal pain. NLR, PLR, neutrophil count and lymphocyte count can be used as attack markers in patients.; however, further studies are needed since this is a single study. NLR and neutrophil count of patients were found to be associated with the number of symptoms in patients with an attack. In our study, R202Q mutation was the 2nd most common genetic mutation unlike other studies. Keyword: Familial Mediterranean Fever, neutrophil/lymphocyte ratio, platelet/lenfosit ratio