Clinical problems and surgical interventions in inherited factor VII deficiency.

aimFactor VII deficiency is one of the hereditary coagulation disorders that has autosomal reccessive inheritance and is observed relatively frequently (1/500 000). It is clinically heterogeneous, and may be asymptomatic or lead to life-threatening bleeding. Thus, there is no correlation between FVII activity and clinical findings. Plasma-derived and recombinant FVII concentrates are currently used for treatment. In countries where access to these products is lacking, fresh frozen plasma and prothrombin complex concentrates are also used, though they contain low amounts of factor FVII. In this study, we present the clinical properties, treatments, and surgical interventions used in patients followed up in our clinic with a diagnosis of factor FVII deficiency. MATERIAL AND

methodsPatients who were diagnosed as FVII deficiency in Division of Pediatric Hematology and Oncology between July 1997 and July 2018, were included in the study. The patients' demographic characteristics, symptoms at presentation, PT, aPTT, and FVII values, types of bleeding, and treatments and surgical interventions used, were recorded. The bleedings observed in the patients were classified by severity as asymptomatic, minor, and major.

resultsA total of 18 patients (7 girls and 11 boys) with a mean age of 9.64±9.63 years were included in the study. The mean follow-up time was found as 78.06±54.4 months. When the bleedings were classified clinically, no bleeding was observed in eight patients (44.4%). The factor FVII level was found as <10% in three of these eight asymptomatic patients and above 20% in the others. Minor bleeding was observed in nine patients (50%) and major bleeding was observed in one patient. When the patients were classified as asymptomatic and symptomatic, there was no significant difference between the two groups in terms of FVII level (p=0.57). A total of 21 surgical interventions were performed in 14 (78%) of 18 patients who were being followed up.

conclusionFVII deficiency has a very wide spectrum both clinically and in terms of approach to surgical interventions. Therefore, patients with factor FVII deficiency should be followed up and treated by comprehensive care centers with close collaboration of multiple disciplines.