1. Introduction Discussion Case report References
Combined factor V and VIII deficiency in a young woman with abundant bleeding after tooth extraction Ihsan Ates1, Mustafa Kaplan1, Gul Tokgoz2, Funda Ceran2, Simten Akalın2, Gulsum Ozet2 1Internal Medicine Department, 2Hematology Department, Ankara Numune Training and Research Hospital, Ankara, Turkey
Haematology
P.0457
Introduction
Discussion
Factor V and VIII deficiency is characterized by normal platelet count and bleeding time with prolonged PT and aPTT . Similarly, total blood count of our patient was normal, double measurements revealed prolonged PT and aPTT (18-20 s and s, respectively), and bleeding time was normal.
In combined factor V and factor VIII deficiency, regular prophylaxis is not required during bleeding episodes and the patient is generally treated only when required, However, in case of recurrent hematoma and hemarthrosis, prophylaxis should be preferred. The treatment of bleeding is the replacement of factor V and VIII. While factor V replacement therapy is done using fresh frozen plasma, factor VIII replacement is done by special factor VIII concentrates (either plasma originated or recombinant factor VIII products). Desmopressin can be given in case of mild bleeding. In our case, the patient was given fresh frozen plasma and her bleeding stopped.
Combined factor V and VIII deficiency, a rare autosomal recessive bleeding disorder, was first defined in 1954 by Oeri et al. Although combined factor V and VIII deficiency is seen all over the world but it is seen in mediterranean countries more frequently. The application is generaly with mild or serious bleeding symptoms such as easy bruising, menorrhagia, epistaxis, gingival bleeding, intramuscular bleeding and operational or after tooth extraction bleeding. Plasma levels of the two factors change between 5 % and 30 %. Treatment of bleeding episodes is done with factor V replacement which is provided by using fresh frozen plasma, desmopressin, and factor VIII replacement provided by using special factor VIII concentrates
Case report
A twenty- year- old woman consulted our polyclinic with the complainment of non- stop bleeding after tooth extraction. From the patient’s history it was learned that she has longlasting bleeding after minor trauma, easy bruising and existing menorrhagia after menstrual siklus going on. Such bleeding findings like melena, hematochezia, haemoptysis, hemarthrosis, petechiae,ecchymosis didn’t exist.
The physical examination and vital findings of the patient whose mother and father was not relatives was normal. Total blood count was normal and prothrombin time (PT) and activated partial prothrombin time (aPTT) values counted twice was respectively and (table.1) (normal referance range for PT: 10,4- 13,4 s and aPTT: s). Bleeding time was normal. aPTT and PT values were found normal after patients plasma was mixed with normal plasma by 1:1 ratio. Fibrinogen, factor VII, factor IX, factor X and factor XI and von willebrand factor levels of the patient were normal. Factor V and factor VIII levels, respectively 11,7 % and 28 %, were low. With these findings, patient was diagnosed as combined factor V and factor VIII deficiency.
References
Oeri J, Matter M, Isenschmid H, et al. Congenital factor V deficiency (parahemophilia) with true hemophilia in two brothers. Bibl Paediatr 58: p , 1954.
Spreafico, M. and F. Peyvandi, Combined Factor V and Factor VIII Deficiency. Semin Thromb Hemost, (4): p
Mannucci, P.M., S. Duga, and F. Peyvandi, Recessively inherited coagulation disorders. Blood, (5): p
Spreafico, M. and F. Peyvandi, Combined FV and FVIII deficiency. Haemophilia, (6): p