International Journal of Hematology and Oncology
2023, Vol 33, Num 4 Page(s): 095-098
HEREDITARY FACTOR VII DEFICIENCY IN TWO SIBLINGS: TWO DISCRETE CLINICAL PRESENTATION
BAHATTİN TUNÇ1, ALİ AYATA1
Department of Pediatric Hematology, Süleyman Demirel University Faculty of Medicine
Keywords: factor vii, factor vii deficiency, childhood
Factor VII (FVII) deficiency is a rare bleeding disorder with a highly variable hemorrhagic predisposition. It is transmitted as an autosomal recessive and plays an important role in the initiation of blood coagulatton forming a complex with tissue factor which activates FIX, FX and FVII zymogen. Prolonged prothrombin time (PT) with normal partial thromboplastin time (PTT) indicates FVII deficiency. For the definitive diagnosis, the specific plasma FVII level should be investigated. FVII deficiency is expressed in different ways and leads to various clinical pictures. We reported two siblings with hereditary factor VII deficiency whose clinical presentations were different.
BAHATTİN TUNÇ1, ALİ AYATA1
Department of Pediatric Hematology, Süleyman Demirel University Faculty of Medicine
Keywords: factor vii, factor vii deficiency, childhood
Factor VII (FVII) deficiency is a rare bleeding disorder with a highly variable hemorrhagic predisposition. It is transmitted as an autosomal recessive and plays an important role in the initiation of blood coagulatton forming a complex with tissue factor which activates FIX, FX and FVII zymogen. Prolonged prothrombin time (PT) with normal partial thromboplastin time (PTT) indicates FVII deficiency. For the definitive diagnosis, the specific plasma FVII level should be investigated. FVII deficiency is expressed in different ways and leads to various clinical pictures. We reported two siblings with hereditary factor VII deficiency whose clinical presentations were different.